We have had the great pleasure this week to help Alexis, a wonderful 16 year old boy who lives in an orphanage with very devoted caregivers, one or more of whom have visited him every day he has been at King Faisel. His mother is alive, but was too poor to raise him. Alexis had been unable to play with his peers, as he had had significant shortness of breath with exertion. He had kept up with his studies, however, despite his disability. On his screening echo, he had what appeared to be isolated severe tricuspid valve regurgitation (leaking of a right-sided heart valve). However, his labs came back with an hematocrit of 79 (nl is about 40-45) and the oxygen saturation in his arterial blood was very low at 77% (nl 98-100%). We had to search further, for a communication between the right and left heart, which would allow blood returning from the body and, therefore depleted of oxygen, to bypass the lungs and get into his left heart and then to his head and body. To evaluate for this communication, we injected agitated saline into an arm vein and we saw the saline contrast enter the right atrium and, then almost immediately, enter the left heart. We had found the communication between both atria (upper chambers of the right and left parts of the heart). To compensate for the low oxygen saturation in his arterial blood, his body had produced almost 2 x the amount of red blood cells to try and oxygenate his body. We then had to make sure that he did not have what we call Eisenmenger physiology where a hole in the heart initially causes excess flow to the lungs, followed by the development of high and irreversible pressure in the lungs. That high pressure would make the shunt reverse direction, sending unoxygenated blood to the body. Our echo, with a small pulmonary artery, equal to the size of the aorta, and only low velocity leakiness of his pulmonic valve (the valve separating the pumping chamber to the lungs and the main vessel to the lungs) did not support irreversible pulmonary hypertension. However, to make sure, our anesthesia team did the first ever right heart catheterization, passing a catheter from a vein in the neck into the right heart and pulmonary artery. We found a normal pulmonary artery systolic pressure of 20 mmHg. We then extensively consulted among our entire team and, via e-mail, cardiopulmonary experts at a combined adult-congenital heart disease program at Children's and the Brigham and the head of cardiac anesthesia at Children's Hospital. Our anesthesia team, led by Drs. Danny Muehlschlegal and Nelson Thaemert, and our perfusion team (they run the heart-lung bypass machine) came up with a surgical game-plan. This involved taking off units of blood and replacing it with fresh frozen plasma to correct his coagulopathy and lower his hematocrit before putting him on the heart lung bypass machine. There was concern that his baseline high hematocrit would cause him to clot in crucial areas of the heart and brain and also that his coagulopathy would cause him to bleed in other areas. On the pre-op night, Alexis developed a supraventricular tachycardia, rate 166/min on 2 occasions, but we were able to restore normal rhythm with medications. He had no chance without surgery. What a brave soul he is. He made friends with all of the other boys we have done and watched them have surgery and begin to recover, while he patiently waited while we determined his operability, tolerating a tranesophageal echo (where he has to swallow a probe a bit smaller than the size of a quarter, to better visualize his communication between the atria), a right heart cath to determine the pulmonary artery pressure (if elevated, he would not be able to be helped), and multiple visits by our entire team to determine if we could do him. I saw occasional tears from the corners of his eyes, as he looked at his fellow operated-on patients and said "I wish that were me. "We were pretty convinced of what was wrong with him, but we lacked the full heart catheterization, CT angiogram and MRI imaging that would have been available to us in the US. We knew there was a small chance that he could have additional pathology which we had not been able to detect. However, we also knew that our team offered him his only chance to grow up—we had been told that he would not be a candidate to go the the Sudan or India for surgery. Fortunately his heart had the pathology we had predicted and, with the excellent skills of our 2 anesthesiologists, our perfusionists and our two very experienced senior surgeons, Dr. Chip Bolman and Dr. Chuck Edwards, he successfully had his huge atrial septal defect closed and his tricuspid valve replaced with a mechanical valve. His blood immediately turned from blue to red. His breathing tube is now out, he is sitting up and enjoying the fact that he is alive and will feel much better. No one in our group had ever cared for this type of problem before. The orphanage has previously been his salvation and the wonderful staff there have promised to bring him back very frequently for INR checks and medical appointments.
We feel lucky to have them be our partners in restoring Alexis to a full life.
Pat Come, MD, cardiologist
Permission granted for disclosure of information from Alexis, 12.Feb.2012
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