The KD parent symposium 2020 highlighted the most recent advancements in Kawasaki disease research, led by the world’s leading researchers in this field. You can find the full video of the symposium here.
Here are some important take home messages:
Professor Michael Levin: New Horizons in Kawasaki
The exact cause of KD is unknown.
Delayed diagnosis and treatment is one of the most important factors in children with KD suffering coronary artery damage.
Diagnosis is not easy as many clinical features of KD are shared with other diseases
Gene ‘’signature”, based on 13 genes has been found to accurately diagnose and distinguish the disease from other causes of fever.
A 13 gene KD kit is underway and could help improve diagnosis.
Adult life after KD
KD usually presents in children under five years old. Children with KD may require life-long treatment, however, since the acute phase of KD is self-limited, KD diagnosis is often missed. Consequently, individuals reach adulthood unaware of their KD history and face multiple cardiac complications. In the US and Japan alone, more than 14,000 new cases of KD are detected each year, yet no official guidelines exist to assist cardiologists in treating patients with a KD history.
Once an individual’s normal arterial wall architecture has been damaged during the acute, inflammatory phase of KD, the affected segment will always have abnormal characteristics. Characteristics resulting from KD earlier in life can contribute to pathological cardiovascular conditions known as cardiovascular sequelae in adulthood. Growing numbers of young adults are presenting to cardiologists cardiovascular sequelae resulting from KD, including…
Cardiomyopathy (a disease of the heart muscle making it harder for the heart to pump blood around the body).
Myocardial ischemia (insufficient blood supply to the heart tissue).
Myocardial infarction (heart attack).
Proliferation of myofibroblasts.
Inability of the CA to dilate in times where myocardial oxygen demand is greater due to the arterial wall’s abnormal composition and calcification.
Propensity to develop aneurisms in systemic arteries (arteries carrying blood from the heart to the body), and patients may present with symptoms of ischemia including weakness in a limb or extremity. These aneurisms have a high propensity to clot, sometimes completely, though many patients do well and can manage this symptom with low-dose aspirin and statins, avoiding bypass surgery.
The management of cardiovascular sequelae conditions is different from atherosclerotic heart disease but there is little awareness in the adult cardiology community regarding the special challenges posed by cardiovascular sequelae of KD. For example, coronary artery aneurisms which can cause insufficient BP and myocardial ischemia in patients with KD history are associated with high risk of thrombosis (clotting) and stenosis (blocking or narrowing) at aneurism. These aneurisms are often highly calcified. In healthy individuals, narrowing of blood vessels can be treated with acetylcholine which induces vasodilation, however, in patients with cardiovascular sequelae from KD, the endothelium of the coronary artery demonstrates paradoxical vasoconstriction in response to this treatment, and thus treatment with acetylcholine is ineffective.
It is therefore crucial for cardiologists to be able to make retrospective KD diagnosis with their adult patients. Cardiologists should, therefore, be familiar with KD signs and symptoms. It is also advised that doctors question the patient's parents about KD-compatible illness. Imaging studies such as magnetic resonance can also be useful. The detection of calcification of the arterial wall at places of former aneurysms is a sign of KD antecedent. However, since the KD etiology remains unknown, no specific test can be conducted for a retrospective KD diagnosis.
KD disease remains enigmatic. To improve the care of patients with antecedent KD, more systematic studies are necessary to collect data about patient histories and outcomes. This will allow the formulation of a hypothesis about the appropriate care and their test under clinical studies.
Research by Dr. J. Burns, UC San Diego School of Medicine
Pregnancy after KD
Due to the relatively recent discovery of KD and lack of awareness of the disease, research into the effects of the disease during and following pregnancy is scarce and sample sizes in studies are generally small. All cardiovascular sequelae of KD pose risks to the mother during pregnancy, labour and delivery, and medications used to manage these conditions may pose risks to the foetus.
In the most recent study of 10 women with a history of KD, only one experienced an obstetrical complication of post-partum haemorrhage, which was possibly related to premature re-institution of heparin therapy after delivery (1). This highlights the need for more studies into the management of KD and associated cardiovascular sequelae in order to develop evidence-based guidelines for obstetricians to support the mothers and babies affected by KD.
Two women in this study delivered infants that developed KD at two and five months. Both mothers were unaware of the genetic component to susceptibility to KD and were unfamiliar with the characteristic clinical signs of KD because they had no personal memory of their illness in childhood. This underscores the need to improve knowledge of the disease’s genetic risk in people with history of the disease.
The study also reviewed reports of the management, delivery method and outcomes in 52 Japanese women with a history of KD. Obstetrical complications were reported in 9.7% of the deliveries including premature rupture of membranes, preterm labor, and post-partum hemorrhage. Cardiovascular complications were also reported in 9.6% of the women. Of the 72 infants, 63 were delivered at term and were healthy; two infants were delivered prematurely at 33 weeks by caesarean section due to maternal distress; five additional infants were born prematurely; two additional infants had congenital abnormalities consisting of ventricular septal defect (hole in the heart) and agenesis of the corpus callosum.
Watch this space for key updates and publications.
Technology and Tools We saw this and hope more doctors will make use of technology and tools like VisualDx so fewer cases of KD go misdiagnosed. This shows how difficult it can be for doctors to diagnose. “It’s one of those conditions where, if you’re not thinking of it, you’ll miss it. Doctors will say, ‘It’s a virus. It’ll pass’, but it’s one of the few conditions where kids can die of a major heart attack”.
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Kawasaki Parent Symposium
To hear more about the history of Kawasaki Disease, ongoing research, and patient experience - click on the video!