Leading Cardiologist Dr.K R Balakrishnan,Director-Cardiac Sciences, Dr.Nandkishore Kapadia, Senior Cardio Thoracic Surgeon & Dr.Suresh Rao

As Rabindranath Tagore said, “Every child comes with the message that God is not yet discouraged of humanity” and Baby Afifa stands as a testimony to just that. Baby Afifa, a 20 old baby had a very large tumour in one of the chambers of the heart and Fortis Malar hospital today announced the successful removal of the tumour in this new born.Leading Cardiologist Dr.K R Balakrishnan,Director-Cardiac Sciences, Dr. Nandkishore Kapadia, Senior Cardio Thoracic Surgeon & Dr.Suresh Rao,HOD – Cardiac Anesthesia & Cardiac Critical Care performed this rare surgery.

On becoming blue and breathless, 4 day old Baby Afifa was rushed to Fortis Malar hospital where she was diagnosed with a tumour in the heart.The child collapsed since the tumour blocking the right side of the heart led to dangerously low oxygen levels.The procedure involved administering anesthesia which was critical as the baby was just a few days old.

The baby’s body temperature was cooled to 15 degree Celsius and the circulation was stopped and under sus
pended animation the tumour was completely removed.The tumour was a leiomyoma- a benign tumour (commonly found in the uterus of a woman)

For the first time in the world a leiomyoma of the heart has been reported in a newborn.Rightly said,All is well that ends well – the rare surgery that was performed was a success and Baby Afifa has recovered well and is expected to lead a normal life.

GOLI SUPPORT MAKES POSSIBLE NEW LIFE SAVING PROGRAM IN CHENNAI

Recently GOLI has donated two pumps to our colleagues in Chennai so as to facilitate the establishment of a program of mechanical support of the circulation or ECLS (extracorporeal life support) in children and adult patients. Two children have benefited already from the new program. The new program relies on GOLI’s continuous mentorship in order to thrive and mature.

Extracorporeal Life Support-ECMO

Extracorporeal life support refers to a medical therapy used to deliver oxygen through an artificial lung, also called oxygenator, and to support the circulation mechanically through a special pump. The pump and the artificial lung represent a miniaturized form of the heart and lung machine used in open heart surgery operations. Various systems are used to achieve ECLS, but in general, the equipment is not only smaller than that used for open heart
surgery, but it is also transportable in various areas of the hospital, including the emergency room and intensive care unit. The patient is connected to the pump and oxygenator through tubes inserted in an artery and in a vein of the patient (see pictures).

ECMO (extracorporeal membrane oxygenation) is the original form of ECLS, and it used for newborns with respiratory failure that fails to respond to medications. like surfactant, gas therapy, like Nitric Oxide, and artificial breathing with mechanical ventilation. Some of these newborns have other congenital anomalies like diaphragmatic hernia and underdeveloped lungs. In diaphragmatic hernia, some of the baby’s bowels are dislodged in the chest because of lack of a portion of the diaphragm, the muscle that normally separates the chest cavity
from the abdomen. In these babies, one of the lungs is usually underdeveloped, frequently requiring ECMO.

Other indications for ECMO/ECLS are heart failure in children and adults, acute lung infection, H1N1 flu, and keeping the patient alive while waiting for a heart or lung transplant. For all these conditions, the pump maintains adequate circulation and blood pressure. The artificial lung replaces the respiration; meaning that it adds oxygen to the blood and it removes carbon dioxide. The patient is supported mechanically until his/her own heart and/or lungs recover.

ECLS and ECMO are lifesaving procedures, but they are associated with complications, like infection, bleeding and stroke. Depending on the patient’s diagnosis and physiologic conditions, survival varies from 30% to 85%. In other words, not all patients will survive. However, historically, without ECLS and ECMO, nearly 100% of patients with conditions now treated with mechanical circulation and ventilation would die. With the refinement of equipment and techniques, it is likely that the outcomes will continue to improve in the years to come.

Following is an informational brochure prepared by our nurse specialist, Joanne Russo, RN and used to educate parents and families of Dr Russo’s patients here in the US. GOLI has provided the brochure to Dr Kapadia as well. The brief educational document describes the procedure, its indications, and its risks.

To: Family and Patients, by Joanne Russo, RN
RE: Extracorporeal Life Support (ECLS)

ECLS is a form of therapy that can keep a very ill patient alive, by supporting the heart and/or lung function when a patient’s own heart and/or lung function is inadequate.

The technology of ECLS is similar to the heart-lung bypass techniques used in cardiovascular surgery. Blood drains from the patient through a tube (catheter) placed in a large vein. This blood passes through a circuit and then is pumped through the membrane oxygenator that serves as an artificial lung, putting oxygen into the blood and removing carbon dioxide.

The blood then passes through a heat exchanger that maintains the blood at normal body temperature. Finally, the blood re-enters the body through a large catheter placed in an artery in the neck or other part of the body. Our team uses centrifugal pumps, closed circuits, and special new oxygenators, which we believe provide several advantages over the traditional neonatal ECLS system, called ECMO (extracorporeal membrane oxygenator).

To place someone on ECLS, the surgeon inserts the catheters into the vein and artery through a small incision made in the neck, groin or chest. This procedure is done in the Intensive Care Unit or the OR. At the same time, perfusionists set up the ECLS circuit, priming it with blood that is carefully tested and cross-matched for compatibility with the patient’s blood type. When the patient and the ECLS circuit are connected, the blood must be thinned with heparin, a drug that prevents the formation of clots. Also, during ECLS support the patient’s lungs continue to be ventilated, but with “lung rest settings”. Often, we use special oxygenators and circuit requiring
low heparin levels.

ECLS does not cure lung or heart disease; it merely supports the patient to allow time for the organs to heal.

ECLS course of treatment can extend from three up 30 days depending upon the severity of the patient’s condition. ECLS is discontinued when careful evaluation of lung and /or heart functions shows that oxygenation and carbon dioxide elimination can be supported with a conventional ventilator, and/or cardiovascular function is adequate by clinical observation and echocardiogram.

Bleeding is the most common complication of ECLS. This problem is associated with the use of heparin, a blood thinner, which must be used. Bleeding is most serious when it occurs in the brain as this can cause brain damage or death. If the patient does experience a brain hemorrhage, or stroke, ECLS may have to be discontinued because the continued use of heparin increases the risk of further bleeding in the brain.

Mechanical failure or equipment malfunction is also possible. The ECLS circuit is composed of many components. If the equipment malfunctions, there is the risk of blood loss or the introduction of air into the system, which could cause death or damage to any of the organs, including the brain.

Chances of patient’s survival depend on the cause of the lung/heart disease, the hospital course before ECLS support, and the patient’s condition while on ECLS.

ECLS is started only on patients who have a chance for a healthy survival, but there are several potential long term problems that can occur. These include chronic lung disease and neurological problems. The neurological problems are usually secondary to bleeding complications during ECLS therapy, to low levels of oxygen to the brain during the time prior to ECLS or during ECLS, or to ligation of the major blood vessels to the brain on the right side of the neck. The lung problems and heart problems are usually the result of the initial lung or heart disease or the lung injury associated with mechanical ventilation.

Lately, the problem of global health has been attracting significant attention in academic centers across the United States. Soon, Duke University and the University of California at San Francisco will launch a Master of Science in Global Health degree program, and the University of California will establish a School of Global Health. Such new interest in global health has been triggered by the high profile activities of celebrities like Bono, George Clooney, Oprah Winfrey, and Angelina Jolie; the work of private foundations, like the Bill & Melinda Gates Foundation; and by the US government grants in support academic initiatives dedicated to global health.

Gift of life International is a private non for profit organization, that has been working in global health since 1992. Specifically, we have been assisting physicians, nurses and hospitals improving care for children with heart disease in various parts of the world, like Latvia, Colombia, Romania and India. In few months, we will embark in a new venture in Kenya.

As outlined in the following blogs by Dr Kumar and Dr Kapadia, outside of South Africa, Africa has an extreme paucity of cardiovascular centers, particularly those specialized in pediatric care. The upcoming program will be a shared venture, the first of its kind, between our Indian colleagues and the GOLI team. The program is being conceived on the premises that India is now an educational, financial, commercial and service hub for a large number of Asian Nations and for East Africa. It is only logic that GOLI would take advantage of our Indian colleagues’ knowledge and their professional network in East Africa to launch our first program in Africa. We believe that this partnership between GOLI and the Chennai’s group represents a smart use of our limited human and financial resources.

As usual, Kenya was chosen because the GOLI team has been invited by the local physicians and by our Indian colleagues who have already had some initial involvement there. In the writings that follow, Dr Kapadia and Dr Kumar illustrate the medical needs in that part of Africa and their initial interactions with the local cardiology team. They report that the hospital structure is modern, but that the expertise in pediatric cardiology and surgery is limited. Most of the equipment at this time is geared towards adult care, which has been the case at the inception of every GOLI’s program since 1992.

The GOLI’s team and I will continue our due diligence in preparation for the first trip to Nairobi, Kenya, planned for February/March 2011. Our involvement in Kenya will not end our commitment in Chennai. Our work in Chennai is moving in new more advanced directions: neonatal surgery, transplantation, mechanical support of the circulation and a formal training program for non Indian specialists. Indeed, we hope that we will be able to establish a formal advanced training program for surgeons and cardiologists from developing Countries, other than India. The Center in Chennai now has the scope and the scale to achieve such ambitious goal, provided that we will continue to assist in the foreseeable future. The expectation is that at the end of the training, the trainees will return to their home Country to develop their own program with external assistance. Again, we believe that this is the most efficient way of using our limited resources.

I am sure that you will enjoy reading Dr Kumar and Dr Kapadia reports on their initial experience in Nairobi.

India is the seventh largest and second most populated Country in the world (population,one billion) and enjoys a history dating back 7,000 years. Currently, India is an emerging economic power, where contrast between old and new is evident at every corner.

India still faces formidable challenges, especially poverty, educational and health care issues. Tuberculosis, HIV/AIDS, malaria and cardiovascular disease are now the primary causes of death thought India. The health care system is based on government hospitals and clinics and a rapidly growing private sector. Ayurveda, the traditional medicine and science based on religious beliefs is still practiced in some areas.

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“What India lacks and the West has is expertise in local clinics to help patients recover”

Simply operating on children with holes in their hearts in India is not enough for Dr. Pierantonio Russo. Ensuring successful surgery means raising standards of healthcare across the board. Dr. Russo, who has 20 years of experience in adult and paediatric cardiac surgery, talks to Sarah Hiddleston about how developing countries can avoid the healthcare learning curve and about the future of heart treatment.

Niabla, 8, sits next to her bed, a cravat of bandages stretching into her shirt. Clutching a small pink heart-shaped pillow, she waves at the man who came from the U.S. to mend her heart for free three days ago, after she was transferred from the Institute of Child Health Egmore to Lifeline Speciality Hospital. “I’m feeling fine. No pain today,” she chirrups.

The pillow is nice for the kids but it has another function — they use it to cough against, a painful but important exercise after surgery. Dr. Russo demands high standards, not just from surgeons, but nurses, juniors, right down to cleaners.

“If you are doing paediatric surgery, everything has to be up to the mark, be it protocols, cleanliness, post-operative care, nutrition or anything else” said Dr. Russo, whose experience encompasses stints at Great Ormond Street, U.K., and the Mayo Clinic, U.S. as well as ‘missions’ in Latvia, Romania and Colombia.

That’s why he believes that his expertise in healthcare management will in time serve more purpose than his surgery.

“India has well-trained physicians and nurses … My role is to help the hospital build the infrastructure that people need and ensure that their standards meet international standards,” said Dr. Russo of the long-term partnership he has with LifeLine.

In his opinion, one way to jump the healthcare learning curve in India is to avoid replicating the insurance-led private healthcare system of the U.S. or the social security system of the U.K. Instead, private and public hospitals could compete for patients who can choose to undergo treatment funded at a fixed rate by the government in a private hospital — a practice recently introduced in his country of birth Italy, which is bringing results.

What India lacks and the West has is expertise in local clinics to help patients recover, so he encourages longer ward stays and social worker visits.

Also, important is investment in stem cell research, which he says is the future treatment for acute and chronic illnesses. IIT (Madras), the LifeLine group, and Dr. Russo, who is currently undertaking a year’s sabbatical to pursue research at Temple University, Philadelphia, are hoping to create beating cells from fibroblasts and insert them into damaged hearts. It is still in the experimental stage but it could avoid the need for Niabla and her ward mates to undergo the trauma of surgery at a relatively late stage.

The children will all go home in a few days and Dr. Russo will return to the U.S. But the lessons learnt, Dr. Russo hopes, will have a domino effect inside and outside the hospital.

The greatest gift of all…

Milena peeks shyly around her mother as the doctors pore over her medical history and test results. She is only three years old, but Milena is living on borrowed time. The tiny girl was born with two holes in her heart: each day, her heart and circulatory system have to work a little harder to overcome the deficiency. She needs a gift few can give her- the gift of life.

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A Heart Lung Bypass Machine

Perfusion as related to cardiac surgery can be defined as the movement of oxygenated blood through blood vessels to vital organs of the body.  Under normal circumstances the heart is the pump which propels the blood to the lungs, where oxygen and CO2 are exchanged.  Blood enriched of oxygen and depleted of CO2 is then pumped to the body’s organs in order to keep them functioning properly.  In cases where the heart has a defect and cannot perform the function of pumping blood, cardiac surgery must be performed to correct the congenital defect.  In order to perform this type of surgery the heart must be stopped so that the cardiovascular surgeon can open the heart and repair the defect.  Therefore the perfusionist is responsible for providing mechanical perfusion for the patient while the heart is stopped.  Utilizing a complex piece of equipment, the heart and lung machine (HLM), the perfusionist can pump oxygenated blood to the patient’s organs temporarily taking place of both the heart and lungs.  This is called placing a patient on cardiopulmonary bypass (CPB).

In order to establish CPB the surgeon places cannulas or tubes into the heart which can be connected to the HLM.  After connection to the HLM the perfusionist essentially drains the blood before it enters the heart into a sterile reservoir where it is pumped through a “manufactured lung” called oxygenator to enrich the blood with oxygen.  The oxygenated blood is then returned to the patient at predetermined calculated flow rates that are comparable to the patient’s normal circulation.  This mechanical perfusion allows the surgeon to operate on the heart since all blood is diverted from the heart to the HLM.

During the majority of heart operations, the heart is not beating and it is not receiving blood and nutrition.  To accomplish this, the surgeon injects a cold medicated solution directly into the aorta or into the coronary arteries until the heart temporarily stops.  The temporary electrical and mechanical inactivity, together with cold temperatures, hypothermia, reduces the metabolism of the heart, protecting it during the time when it does not receive blood and oxygen.

Certain defects require the perfusionist to mechanically control temperature as well.  On all routine cases the blood is warmed to normal human temperature and is held there.

Since children are so small and the operating room is cold combined with their open chest, heat loss occurs.   However, perfusionist can counteract this using warming systems connected to the HLM.  In more complex cases where the surgery may be long and involved perfusionist will cool the patient to lower temperatures to protect the organs against injury.  This can be as extreme as where the child is cooled to the point where the HLM can be turned off for a period of time to allow the surgeon to remove the tubing so that he can see the smallest of structures found in the patient’s heart to repair.  Some may have heard stories about how children who fall into icy water in winter time can be resuscitated hours later.  Perfusionist use this same mechanism with cooling since it protects vital organs and limits the necessity for oxygenated blood to some degree.

Perfusionist must make split second decisions and must constantly maintain vigilance while the patient is on CPB since the perfusionist who is operating the HLM is maintaining the patient’s life mechanically while the surgeon operates.  The perfusionist performs many tests throughout the procedure to ensure that the organs are receiving the correct amounts of oxygen and nutrients carried in the blood.  The perfusionist also monitors blood clotting which can be detrimental to the patient if certain parameters are not met.  Heparin is used to “thin” the blood so that it flows easily through the HLM and does not clot.  This test is performed many times throughout the procedure as it is critical to successful surgery on bypass.

After the surgeon repairs the defect the patient if cold, is re-warmed to normal temperature and then the heart begins to beat once the infusion of the heart stopping solution is terminated.  As the heart “wakes” up the perfusionist corrects problems with

the blood oxygenation or nutrients and prepares to terminate bypass.  To terminate bypass the perfusionist slowly restricts the amount of blood returning to the reservoir and allows the heart to become full and begins pumping the blood to the body.  As more blood is returned to the heart to pump, the perfusionist decreases the flow of the HLM until the heart is completely pumping and oxygenating blood on its own.  At this point CPB is terminated and the cannula can be removed from the heart and thinning of the blood can be reversed using another medication called protamine.  The duration of CPB is based on the type of defect being repaired and complexity of the anatomy.

Perfusionist are credentialed in The United States by the American Board of Cardiovascular Perfusion, and obtains the Certified Clinical Perfusionist or CCP after completion of an accredited program in perfusion as well as a board examination.  Other countries have different requirements but most have formal training schools and testing.  All perfusionists work under the direction of a cardiovascular surgeon and communication between the surgeon and perfusionist is crucial throughout the entire surgery.

Most perfusionists are clinically involved with adult patient populations but there are also perfusionists which are affiliated with children hospitals and only perform CPB for children.  Perfusionist who travel with the Gift of Life, Inc. are all specialized in pediatric congenital heart surgery and bring a wealth of knowledge from different regions of the US.  Along with the other members of the open heart team perfusionist are a critical component to successful congenital open heart surgery.

Brian C. Forsberg, BS CCP-LP

Director of Perfusion

Dragos now has a bright future ahead of him and this is what our son is today and it is all thanks to you.

God bless you for what you are doing, and always realize that you bring smiles on other people’s faces and tears of joy in other grateful eyes.

Eternal gratitude,
Dragos, Marcel and Geta Tuluc, from Cluj-Napoca, Romania