奥村 謙一^{1, 2}, FRIEDBERG Mark²

Kenichi OKUMURA^{1, 2}, Mark FRIEDBERG²

¹京都府立医科大学小児循環器・腎臓科, ²Echocardiography, The Hospital for Sick Children, Toronto

¹Pediatric Cardiology and Nephrology, Kyoto Prefectural University of Medicine, ²Echocardiography, The Hospital for Sick Children, Toronto

キーワード :

【Background】
One important thing in children with pulmonary arterial hypertension（PAH）with congenital heart disease is existence of an intracardiac shunt. We investigated echocardiographic parameters of right ventricular（RV）performance in children with PAH with or without an intracardiac shunt in relation to simultaneously measured invasive reference measures.
【Methods】
We prospectively recruited sixteen children undergoing catheterization for evaluation of PAH. Echocardiography was performed simultaneously with catheterization.
【Results】
Seven patients（44％）with an intracardiac shunts who did not undergo surgical repair were allocated to the shunt group. Nine patients（56％）with a structurally normal heart or with operated intracardiac shunts were allocated to the non-shunt group.
Systolic function- Significant correlations were found between catheter dP/dT maximum and tricuspid regurgitation derived dP/dT maximum and tricuspid isovolumic acceleration. Indexed pulmonary blood flow correlated significantly with isovolumic contraction time, tissue Doppler Tei index, longitudinal strain and strain rate（SR）.
Diastolic function- Significant correlations were found between tau and tricuspid deceleration time, E’，E/E’，TimeE-E’，A wave velocity and early and late diastolic SR. Catheter dP/dT minimum correlated significantly with A’，and late diastolic SR.
Correlations between echocardiographic and catheter parameters were more prevalent and robust in children without an active intracardiac shunt.
【Conclusion】
Echocardiography correlates with invasive reference measures of RV performance in children with PAH, but we should pay attention with existence of intracardiac shunt when we measure echocardiographic RV diastolic parameters in children with PAH.