Conference: 2012 International PHA Conference and Scientific Sessions
Release Date: 06.22.2012
Presentation Type: Abstracts
BACKGROUND: Kawasaki Disease (KD) is an acute, self-limited vasculitis marked by significant systemic inflammation and cardiovascular manifestations including carditis, coronary artery aneurysm, and endothelial dysfunction. There is increasing awareness of the role of inflammation in pulmonary hypertension (PH) however PH has not been described in the setting of KD.
METHODS: We present a case series of two pediatric patients diagnosed with KD with echocardiographic findings consistent with PH.
RESULTS: Patient 1 is a 4 year-old male diagnosed with typical KD by classical diagnostic criteria (>5 days of fever, diffuse erythematous macular rash, bilateral pedal and palmar edema, scleral and conjunctival injection, oral mucosal inflammation, lymphadenopathy, and elevated inflammatory markers). His echocardiogram at the time of diagnosis demonstrated a peak tricuspid regurgitant (TR) gradient of 40 mmHg consistent with an estimated pulmonary artery systolic pressure of 45 mmHg. Left ventricular (LV) function, coronary arteries, and the remainder of the echocardiogram were within normal limits. After treatment with 1 dose of IVIg and 1 week of high-dose Aspirin followed by 3 months of low-dose Aspirin, he had normalization of his TR gradient to 17 mmHg.
Patient 2 is a 14 year-old male diagnosed with incomplete KD (>5 days of fever, bilateral pedal and palmar edema which progressed to a desquamating rash, diffuse erythematous macular rash, pulmonary infiltrates, elevated transaminase levels and hyperbilirubinemia with scleral icterus, and elevated inflammatory markers). Initial echocardiogram at diagnosis showed qualitatively moderately depressed right ventricular function and peak TR gradient of 59 mmHg. LV function, coronary arteries, and the remainder of the echocardiogram were normal. Computed tomography of the chest was negative for pulmonary embolus (PE). After treatment with 2 doses of IVIg and high-dose Aspirin for 2 weeks followed by 4 weeks of low-dose Aspirin, his right ventricular (RV) function and TR gradient normalized.
CONCLUSIONS: PH is a previously unreported complication of KD. Of note, cardiac catheterization was not performed in either of these patients due to the rapid resolution of the apparent PH so it is not possible to be certain of their hemodynamics. Neither patient had echocardiographic evidence of LV systolic or diastolic dysfunction. A high cardiac output due to a systemic inflammatory response may have contributed to elevated RV pressure but this would be unlikely to account for the significant elevation in RV pressure, especially in patient 2. We considered acute PE as an explanation in patient 2 though literature suggests that there is not a hypercoaguable state in acute KD. We hypothesize that KD can result in pulmonary vasculitis and endothelial dysfunction leading to an acute elevation in pulmonary vascular resistance and pulmonary artery pressure. Acute PH appears to be a rare complication of KD which responds promptly to conventional treatment with IVIg and high-dose aspirin.