Author + information
- John B. Gordon, MD* (, )
- Andrew M. Kahn, MD, PhD and
- Jane C. Burns, MD
- ↵*San Diego Cardiac Center, Sharp Memorial Hospital, 3131 Berger Street, Suite 200, San Diego, California 92123
We thank Drs. Gersony and Dahdah for their thoughtful comments regarding our paper (1) on Kawasaki disease (KD) in the adult patient. There are several issues worthy of further discussion. In his perspective, Dr. Gersony agrees that the small minority of patients with giant aneurysms are at high risk for future cardiac events and deserve long-term follow-up as recommended by the American Heart Association guidelines. We suggested that “on the basis of the accumulating evidence, it is likely that patients with known aneurysms during the acute phase of KD will have some cardiovascular morbidity as young adults.” Dr. Gersony responded that this is “possibly true for patients with large aneurysms but not evidence based for patients with small aneurysms.” Unfortunately, the current published literature on this issue is incomplete, and the long-term outcomes for the patients in question are not really known. The majority of adults with KD will do well, but patients discharged by pediatric cardiologists with aneurysms that have “normalized” by transthoracic echocardiography have presented in our emergency room with heart failure related to severe left ventricular dysfunction. Perhaps, as Dr. Dahdah suggests, “the myocarditis should not be underestimated.” Indeed, the myocardial fibrosis resulting from myocarditis associated with acute KD may turn out to be as problematic as the ischemic events related to the coronary artery disease. At this time, we simply do not have the answers to many key questions. We look forward to adequately powered, prospective, longitudinal cohort studies that will establish the numerator and denominator for the cardiovascular risk equation after KD. These studies must track not only coronary artery sequelae but also myocardial systolic and diastolic function, myocardial perfusion, valvular function, aortic root dimensions, and endothelial cell function.
We appreciate Dr. Gersony's thoughtful perspective as a senior pediatric cardiologist and agree that the majority of patients with KD fully recover from their acute vascular and myocardial insult suffered in childhood. We respectfully disagree, however, with his opinion that only patients with giant aneurysms need to be followed up longitudinally. Does the vascular injury in childhood make coronary arteries more prone to atherosclerosis and ischemic complications if the adult smokes or has diabetes? Once again, Dr. Gersony correctly observes that “there is no direct evidence” that this is the case. The absence of systematically collected data does not guarantee the absence of important problems. As adult cardiologists, we have seen young adults post-KD in childhood present with severe coronary artery disease in modest aneurysms as well as in vessels without previous aneurysms. The natural history of the vascular injury caused by KD in childhood has not yet been defined. Dr. Gersony states that it is important to avoid creating “patients” among persons who have not been shown to be at increased late risk for a cardiac event. It has been our experience that longitudinal follow-up of this patient group provides reassurance to patients and to their families rather than creating anxiety or concern.
Few adult cardiologists have managed patients with complications of KD. Failure to recognize these patients is a major problem. Our review was written with the intention of raising awareness about this remarkable disease. The eyes cannot see what the mind does not know.
- American College of Cardiology Foundation