+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Pulmonary Arterial Capacitance Index Is a Strong Predictor for Adverse Outcome in Children with Idiopathic and Heritable Pulmonary Arterial Hypertension



Pulmonary Arterial Capacitance Index Is a Strong Predictor for Adverse Outcome in Children with Idiopathic and Heritable Pulmonary Arterial Hypertension



Journal of Pediatrics 180: 75-79.E2



To evaluate the clinical utility of pulmonary artery capacitance index (PACi) in the assessment of disease severity and prognostic value in children with idiopathic and heritable pulmonary arterial hypertension (PAH). PACi is defined as the ratio of stroke volume index over pulmonary pulse pressure. A retrospective study was performed to compare PACi, brain natriuretic peptide (BNP), 6-minute walk distance, New York Heart association (NYHA) functional class, and adverse outcomes (hospitalization due to heart failure, lung transplantation, and cardiac mortality) in 72 Japanese children (10 ± 3.6 years) with idiopathic and heritable PAH. PACi had significant correlations with pulmonary vascular resistance index (r =-0.73, P < .0001), BNP levels (r = -0.40, P = .0008), and 6-minute walk distance (r = 0.57, P < .05). Statistically significant differences in PACi were observed between NYHA functional class II vs combined III and IV (median; 1.1 vs 0.6 mL/mm Hg/m2, respectively, P < .05). There were 25 of 72 (35%) children who had an adverse event including initiation of hospitalization due to heart failure, lung transplantation, and death. Cumulative event-free survival rate was significantly lower when PACi was <0.85 mL/mm Hg/m2 (log-rank test, P < .0001). PACi correlated with BNP and NYHA functional class and may serve as a strong prognostic marker in children with idiopathic and heritable PAH.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 058662429

Download citation: RISBibTeXText

PMID: 27810156

DOI: 10.1016/j.jpeds.2016.10.003


Related references

Pulmonary arterial capacitance index is a strong predictor for adverse outcome in children with idiopathic and heritable pulmonary arterial hypertension: methodological issues to avoid misinterpretation. Journal of Pediatrics 184: 239-240, 2017

Pulmonary arterial capacitance in children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease: relation to pulmonary vascular resistance, exercise capacity, and survival. American Heart Journal 162(3): 562-568, 2011

Pulmonary Vascular Capacitance Is A Strong Predictor Of Mortality In Patients With Idiopathic And Scleroderma-Associated Pulmonary Arterial Hypertension. Chest 132(4): 636-1, 2007

Prolonged QRS duration: a new predictor of adverse outcome in idiopathic pulmonary arterial hypertension. Chest 141(2): 374-380, 2012

Pulmonary Vascular Capacitance as a Predictor of Vasoreactivity in Idiopathic Pulmonary Arterial Hypertension Tested by Adenosine. Research in Cardiovascular Medicine 4(4): E28945, 2015

Clinical Effects of Syncope on Disease Severity and Adverse Outcomes in Children with Idiopathic and Heritable Pulmonary Arterial Hypertension. Pediatric Cardiology 2018, 2018

Long-term Outcomes of Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease as Compared With Idiopathic or Heritable Pulmonary Arterial Hypertension Treated With Tadalafil. Chest Journal 140(4): 734a-734b, 2011

Clinical Impact of Main Pulmonary Artery Dilatation on Outcome in Pediatric Idiopathic and Heritable Pulmonary Arterial Hypertension. Circulation Journal 82(2): 541-545, 2017

Relationship of pulmonary arterial capacitance and mortality in idiopathic pulmonary arterial hypertension. Journal of the American College of Cardiology 47(4): 799-803, 2006

Relationship of Pulmonary Arterial Capacitance and Mortality in Idiopathic Pulmonary Arterial Hypertension. Yearbook of Cardiology 2007: 411-412, 2007

Tissue Doppler imaging predicts adverse outcome in children with idiopathic pulmonary arterial hypertension. Journal of Pediatrics 161(6): 1126-1131, 2013

Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. Journal of Heart and Lung Transplantation 24(10): 1626-1631, 2005

C-reactive protein: a new predictor of adverse outcome in pulmonary arterial hypertension. Journal of the American College of Cardiology 53(14): 1211-1218, 2009

The Recent Prognosis and Treatment of Idiopathic and Heritable Pulmonary Arterial Hypertension; The Report from Single Pulmonary Hypertension Center in Japan. Journal of Heart and Lung Transplantation 36(4): S362-S363, 2017

Current epoprostenol use in patients with severe idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension: data from the French pulmonary hypertension registry. International Journal of Cardiology 172(3): 561-567, 2015