Melle D. Talsma

Acute pulmonary vasodilator response in paediatric and adult pulmonary arterial hypertension : occurrence and prognostic value when comparing three response criteria

AIMS To assess the occurrence and prognostic value of acute vasodilator response (AVR) in paediatric vs. adult pulmonary arterial hypertension, and idiopathic/hereditary pulmonary arterial hypertension (iPAH/HPAH) vs. pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) using three different response criteria. METHODS AND RESULTS Ninety-nine PAH patients underwent AVR testing (37 children, 62 adults; 70 iPAH/HPAH, 29 PAH-CHD). Three response criteria from clinical practice were used to define AVR. The number of responders was evaluated separately in subgroups based on age, diagnosis, and presence of a non-restrictive post-tricuspid shunt. Numbers of responders varied importantly using the different criteria but were always higher in iPAH/HPAH, compared with PAH-CHD. The number of responders did not differ between paediatric and adult iPAH/HPAH. No responders were identified in patients with a post-tricuspid shunt. Acute vasodilator response was associated with improved survival using all three criteria. Low baseline mean right atrial pressure (mRAP) was associated with improved survival in adults (P< 0.001). High baseline mean pulmonary arterial pressure (mPAP)/mean systemic arterial pressure (mSAP) and pulmonary vascular resistance (PVR)/systemic vascular resistance (SVR) were associated with worse survival, statistically independent from age, diagnosis, and the presence of a post-tricuspid shunt. CONCLUSION The proportion of patients with AVR highly depends on the used criteria, but did not differ between paediatric and adult iPAH/HPAH. Current response criteria are not suitable for patients with a post-tricuspid shunt. In both children and adults without post-tricuspid shunts, AVR was associated with improved survival independent of the used criteria. Nevertheless, prognostic value in the individual patient was limited. Baseline mRAP showed a good correlation with survival for adult PAH patients, but not for children. High baseline mPAP/mSAP and PVR/SVR was associated with worse prognosis independent from age, diagnosis, or the presence of a post-tricuspid shunt.

Pulsatile haemodynamic parameters are predictors of survival in paediatric pulmonary arterial hypertension

BACKGROUND There is a need for reliable prognostic parameters in pulmonary arterial hypertension (PAH), especially in children. Pulsatile components of the right ventricular afterload, represented by pulmonary arterial compliance (PACi) and pulmonary stroke volume (PSVi), may provide important additional prognostic information to conventional static haemodynamic parameters. The aim of this study was to determine the prognostic value of PACi and PSVi in paediatric PAH. METHODS Right heart catheterization data of 52 consecutive paediatric idiopathic/hereditary PAH and PAH associated with congenital heart disease patients with full haemodynamic evaluation seen at the Dutch national referral centre for paediatric pulmonary hypertension between 1993 and 2010 were reviewed. A control group was composed of patients with normal pulmonary vascular resistance. PSVi and PACi were calculated and tested for predictive value for transplant-free survival. RESULTS PAH patients had significantly lower PSVi and PACi compared to control patients. PSVi and PACi were lower in patients with higher WHO-functional class compared to those with lower functional classes. Higher PSVi, PACi and mSAP and lower mPAP/mSAP and heart rate were associated with improved survival, independent from WHO-functional class and PAH-targeted therapy. In multivariate analyses PSVi, heart rate and mSAP emerged as the strongest haemodynamic predictors of survival. The effect of vasodilator challenge on the haemodynamic variables did not provide additional prognostic information. CONCLUSIONS The parameters of both the pulsatile and static pulmonary circulations are strong independent predictors for transplant-free survival, and therefore can be of complementary value in assessing disease severity, predicting survival and guiding treatment in paediatric PAH.

AORTOPULMONARY WINDOW ASSOCIATED WITH AN ANOMALOUS ORIGIN OF THE RIGHT CORONARY-ARTERY

Abstract We report a case of a large aortopulmonary window associated with an anomalous origin of the right coronary artery from the pulmonary trunk and discuss the etiology of this rare anomaly.

Pulse Densitometer Indocyanine Green Dilution Curves: A Simple Applicable and Accurate Method for Determination of Cardiac Shunts

OBJECTIVE Adequate hemodynamic evaluation is crucial in the management of patients with congenital heart disease. Although non-invasive diagnostic tools have reduced the need for invasive procedures, cardiac catheterization is still mandatory for absolute quantification of pressures, flows and vascular resistances in selected patients. We therefore investigated the feasibility of a new technique, non-invasive pulse densitometry (PD) in patients with intracardiac shunts and compared its results with the established standards: cuvette densitometry (CD) and oximetry (OX). DESIGN Measurement method comparison study. OUTCOME MEASURE In 12 patients with intracardiac shunt, dye dilution curves, using both pulse and cuvette densitometry, were recorded and oximetry was performed. Left-to-right shunt expressed as percentage of pulmonary blood flow Qp, was calculated from dye dilution and oximetry. In 4 patients with atrial septal defect, dye dilution curves were also recorded after closure of the defect with a device. RESULTS The mean difference +/- SD between the shunt derived from PD and CD was 2.8 +/- 10.0% of Qp, 95% confidence interval -2.5 to 8.2. (Shunt-PD vs. Shunt-CD was 32.3 +/- 23.9% vs. 29.5 +/- 23.9% of Qp resp., n = 16). The mean difference +/- SD between the shunt derived from PD and OX was 0.8 +/- 9.8% of Qp, 95% confidence interval -5.4 to 7.0 (Shunt-PD vs. Shunt-OX was 41.5 +/- 20.3% vs. 40.7 +/- 19.7% of Qp resp., n = 12). CONCLUSION Transcutaneous recording of dye dilution curves with a pulse dye densitometer allows easy and accurate quantification of intracardiac left-to-right shunt flows over a wide range in both children and adults with congenital heart diseases.