Pulmonary Artery Dilation and Right Ventricular Function in Acute Kawasaki Disease

Abstract

Coronary artery inflammation and aneurysm formation are the most common complications of Kawasaki disease (KD). Valvulitis and myocarditis are also well described and may lead to valvar regurgitation and left ventricular dysfunction. However, functional changes in the right heart have rarely been reported. We noted several acute KD patients with dilated pulmonary arteries (PA) and thus sought to systematically characterize PA size and right-heart function in an unselected cohort of KD patients cared for at a single clinical center. Clinical, laboratory, and echocardiographic data from 143 acute KD subjects were analyzed. PA dilation was documented in 23 subjects (16.1 %); these subjects had higher median right ventricle myocardial performance index (RV MPI), higher ratio of early tricuspid inflow velocity to tricuspid annular early diastolic velocity (TV E/e'), and lower median TV e' velocity compared to the non-PA dilation group (0.50 vs 0.38 p < 0.01, 4.2 vs 3.6 p < 0.05, and 13.5 vs 15.2 cm/s p < 0.01, respectively). Almost all subjects with PA dilation had improved PA Z-score, RV MPI, and TV E/e' in the subacute phase (p < 0.01). There were no significant differences in indices of left ventricle function between PA dilation group and non-PA dilation group. In summary, PA dilation was documented in 16 % of acute KD subjects. These subjects were more likely to have echocardiographic indices consistent with isolated RV dysfunction that improved in the subacute phase. The long-term consequence of these findings will require longitudinal studies of this patient population.

Bifid T waves on the ECG and genetic variation in calcium channel voltage‐dependent beta 2 subunit gene ( CACNB2 ) in acute Kawasaki disease

“…Clinically, LV dysfunction presumably related to myocardial inflammation has been described in series of patients with “KD shock syndrome.” One case report showed findings of myocardial edema and decreased LV function by cardiac magnetic resonance imaging . Numano et al reported the existence of RV dysfunction in acute KD documented by echocardiography . Therefore, myocardial inflammation in both the right and left heart in acute KD may lead to dispersion of ventricular repolarization and bifid T waves.…”

“…2,25,26 Clinically, LV dysfunction presumably related to myocardial inflammation has been described in series of patients with "KD shock syndrome." 29 Therefore, myocardial inflammation in both the right and left heart in acute KD may lead to dispersion of ventricular repolarization and bifid T waves. 28 Numano et al reported the existence of RV dysfunction in acute KD documented by echocardiography.…”

“…28 Numano et al reported the existence of RV dysfunction in acute KD documented by echocardiography. 29 Therefore, myocardial inflammation in both the right and left heart in acute KD may lead to dispersion of ventricular repolarization and bifid T waves.…”

Dual-source Computed Tomography for Evaluating Pulmonary Artery and Aorta in Pediatric Patients with Single Ventricle

“…3 ). Using the mean diameters of MPA and AAO of control subjects as a contrast standard, the DSCT-based diameters of both arteries in patients were compared with the control values: MPA stenosis was defined as <75% contrast standard 3 , 23 and MPA dilation was defined as Z-score (standard deviation units from the mean) >2 24 . AAO dilation and stenosis were regarded as Z-score > 2 and Z-score < −2, respectively 25 .…”

Lipocalin-2-induced proliferative endoplasmic reticulum stress participates in Kawasaki disease-related pulmonary arterial abnormalities

“…Notably, pulmonary arterial hypertension (PAH), pulmonary artery dilation, pulmonary artery aneurysms, and right ventricle (RV) dysfunction have been observed in patients with KD (Briceno-Medina et al, 2016;Escalon et al, 2018;Nicholson et al, 2013;Ugi et al, 2010). Furthermore, a remarkably lower pulmonary arterial compliance and diffused histologic changes in pulmonary arteries are also observed in patients with KD (Masuda et al, 1986;Numano et al, 2016;Sugimoto et al, 2012;Takahashi K., 2017). However, the underlying mechanisms responsible for these pulmonary arterial abnormalities in KD are still unknown and need further investigation.…”