Invasive hemodynamics as predictor of outcomes in pediatric Pulmonary Arterial Hypertension
Presenter
Rupesh Kumar Natarajan, MD, University of Minnesota Masonic Children's Hospital, Minneapolis, MN
Rupesh Kumar Natarajan, MD1, Nathan J Rodgers, MD1, Matthew Ambrose, MD1, Shanti Narasimhan, MD, FASE1, Abraham Rothman, M.D., FSCAI2 and Varun Aggarwal, M.D.3, (1)University of Minnesota Masonic Children's Hospital, Minneapolis, MN, (2)Children's Heart Center Nevada, Las Vegas, NV, (3)The University of Minnesota, Twin Cities, Minneapolis, MN
Keywords: Congenital Heart Disease (CHD)
Background
Pulmonary arterial hypertension (PAH) can lead to significant morbidity and mortality. Invasive hemodynamics is often performed for guiding PAH therapy. Little is known about the predictive value of the invasive hemodynamic indices in children with PAH. We aimed to evaluate invasive hemodynamic data to predict outcomes in pediatric PAH.
Methods
Patients with PAH who underwent cardiac catheterization (2006-2019) at the University of Minnesota Masonic Children’s hospital were included. Invasive hemodynamic data from the first cardiac catheterization and clinical outcomes were obtained. Combined adverse outcome was defined as creation of an atrial or reverse Pott’s shunt, death, lung transplant, pericardial effusion. Univariate logistic regression and receiver operating characteristic (ROC) analysis were performed.
Results
Out of 49 patients (59% males) with median (IQR) age of 12.8 (4.2, 48.7) months, 71% had congenital heart disease. At the time of catheterization, median BNP was 2520 ng/L (n=29) and 39% patients were on at least one PAH medication. Median pulmonary artery pulsatility index (PAPi) was 3.75 (2.67,4.71) and indexed RV stroke work (RVSWi) was 704 mmHg mL/m2 (532-1151). PA elastance ≥0.94 and PA compliance ≥1.35 ml/mmHg predicted a baseline NT pro-BNP >2145 ng/L (p<0.05). After median follow up of 2.5 years, 10 patients had combined adverse outcome (2 underwent creation of an atrial level shunt, 6 died, none had lung transplant and 2 had pericardial effusion). Patient who had combined adverse outcome had higher mean and systolic PA pressures, higher diastolic and transpulmonary pressure gradients, higher PVRi, higher PA elastance and higher RVSWi. (p <0.05 each). Mean PA pressure of >42.5 mmHg had 89% sensitivity (AUC=0.85, p=0.001) and 73% specificity while diastolic pressure gradient >21.5 mmHg had 78% sensitivity and 80% specificity (AUC=0.81, p=0.004) in predicting the combined adverse outcome. PAPi was not a significant predictor of adverse outcome.
Conclusions
Invasive hemodynamics performed at initial cardiac catheterization after diagnosis of PAH may help predict outcomes in pediatric patients. This information may have utility in guiding therapy and counselling families about the outcomes.