Authors' Reply

Abstract

We thank Drs. Koestenberger and Ravekes for their interest in our study1Lammers A.E. Haworth S.G. Riley G. Maslin K. Diller G.P. Marek J. Value of tissue Doppler echocardiography in children with pulmonary hypertension.J Am Soc Echocardiogr. 2012 May; 25: 504-510Abstract Full Text Full Text PDF PubMed Scopus (44) Google Scholar and for adding to the discussion on the value of longitudinal right ventricular (RV) function in children with pulmonary arterial hypertension. We agree with the authors on the utility of Z scores in this population. We calculated Z scores for our data but decided against including them in the study, as we felt that their use would not provide incremental information in the presence of a matched control group. Especially for longitudinal studies and clinical patient follow-up, however, Z scores may be useful. Koestenberger and Ravekes also point to interesting publications in the field of pulmonary hypertension secondary to congenital heart disease. We would like to highlight that the patients included in our study had no histories of pericardiotomy. Previous pericardiotomy has been recently described to affect measures of RV longitudinal function. In fact, using intraoperative transesophageal echocardiography, Unsworth et al.2Unsworth B, Casula RP, Yadav H, Baruah R, Hughes AD, Mayet J, et al. Contrasting effect of different cardiothoracic operations on echocardiographic right ventricular long axis velocities, and implications for interpretation of post-operative values. Int J Cardiol. In press.Google Scholar showed that RV longitudinal function deteriorated within 5 min of full opening of the pericardium. Other potential confounders of RV function in the setting of congenital heart disease include the presence of RV fibrosis as a consequence of previous cyanosis and surgical interventions.3Babu-Narayan S.V. Kilner P.J. Li W. Moon J.C. Goktekin O. Davlouros P.A. et al.Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome.Circulation. 2006; 113: 405-413Crossref PubMed Scopus (451) Google Scholar This, in turn, may also affect the left ventricle.4Hausdorf G. Hinrichs C. Nienaber C.A. Schark C. Keck E.W. Left ventricular contractile state after surgical correction of tetralogy of Fallot: risk factors for late left ventricular dysfunction.Pediatr Cardiol. 1990; 11: 61-68Crossref PubMed Scopus (53) Google Scholar, 5Davlouros P.A. Kilner P.J. Hornung T.S. Li W. Francis J.M. Moon J.C. et al.Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction.J Am Coll Cardiol. 2002; 40: 2044-2052Abstract Full Text Full Text PDF PubMed Scopus (440) Google Scholar Therefore, beyond the obvious parallels between pulmonary hypertension associated with congenital heart disease and other forms of the conditions, pathophysiologic differences exist that should not be ignored. Similar considerations are relevant with regard to diaphragmatic hernia, a condition likely to affect RV function through a combination of lung hypoplasia with subsequently increased pulmonary vascular resistance, dislodgement of the heart, and external compression of the pericardium. Our data suggest that measures of biventricular longitudinal dysfunction may be useful adjuncts in assessing cardiac mechanics, and the interaction between the ventricles might in the future provide further insight into the mechanisms leading to end-stage pulmonary hypertension. We agree with Koestenberger and Ravekes that a comprehensive echocardiographic assessment of biventricular systolic function would be desirable in all pediatric patients with pulmonary hypertension. Further longitudinal studies are required, however, to correlate reduced longitudinal function with clinical deterioration and adverse outcome and to understand how tissue Doppler echocardiography could specifically guide therapy in this challenging population. Value of Tricuspid Annular Plane Systolic Excursion and Peak Systolic Velocity in Children with Pulmonary HypertensionJournal of the American Society of EchocardiographyVol. 25Issue 12PreviewWe read with interest the article “Value of Tissue Doppler Echocardiography in Children With Pulmonary Hypertension” by Lammers et al.1 In our opinion, this is a very interesting report describing right ventricular (RV) dysfunction in children with idiopathic pulmonary artery hypertension (PAH). The investigators clearly stated that there is a need for a detailed evaluation of RV functional parameters such as tricuspid annular plane systolic excursion (TAPSE) in their population to provide further insight into RV dysfunction in this setting and therefore help clinicians optimize treatment for RV failure. Full-Text PDF