Right ventricular remodeling in idiopathic pulmonary arterial hypertension: adaptive versus maladaptive morphology
Section snippets
Population and study protocol
Our study population comprised 60 consecutive therapy-naïve patients affected by IPAH, World Health Organization functional class II to IV, without severe tricuspid regurgitation, referred to our Pulmonary Hypertension Unit (Policlinico Umberto I, Sapienza University of Rome) from January 2011 to December 2012. A control group for RV M/V ratio evaluation comprised 20 healthy subjects matched for age, gender, weight, and height. According to the European guidelines,10 the diagnosis of PAH was
Results
Table 1 summarizes the characteristics of the study population. Most of the patients with IPAH were female and in World Health Organization class III, with severe pulmonary hypertension and impaired effort capacity. Clinical depression was the most frequent co-morbidity, followed by thyroid disease and hypercholesterolemia.
Compared with normal subjects, patients with IPAH showed higher RV mass and volume values, lower RV ejection fraction, and higher RV M/V ratio. Among the IPAH group, RV M/V
Discussion
This study combines echocardiographic and CMR imaging to evaluate the influence of different modalities of RV morphologic adaptation to afterload on RV systolic function, hemodynamics, and clinical status of patients with IPAH. As expected, patients with IPAH showed severe RV remodeling, with higher mass values and larger volumes compared with the control group. Patients with IPAH showed a higher RV M/V ratio compared with healthy subjects. This pattern (higher M/V ratio) suggests that
Disclosure statement
Each author of this article has made substantial contributions to conception and design and acquisition, analysis, and interpretation of data; has drafted the submitted article or revised it critically for important intellectual content; has provided final approval of the version to be published; and has agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
This
References (35)
- et al.
Determinants of right ventricular ejection fraction in pulmonary arterial hypertension
Chest
(2009) - et al.
Progressive changes in right ventricular geometric shortening and long-term survival in pulmonary arterial hypertension
Chest
(2012) - et al.
Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy
J Am Coll Cardiol
(2011) - et al.
Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography. Endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography
J Am Soc Echocardiogr
(2010) - et al.
An echocardiographic index for separation of right ventricular volume and pressure overload
J Am Coll Cardiol
(1985) - et al.
Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension
J Am Coll Cardiol
(2002) - et al.
Long-term intravenous epoprostenol infusion in primary pulmonary hypertension. Prognostic factors and survival
J Am Coll Cardiol
(2002) - et al.
The right ventricle under pressure. Cellular and molecular mechanisms of right heart failure in pulmonary hypertension
Chest
(2009) - et al.
Right ventricular ischemia in patients with primary pulmonary hypertension
J Am Coll Cardiol
(2001) - et al.
Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome
Am J Cardiol
(2002)
Inhaled nitric oxide versus prostacyclin in chronic shunt-induced pulmonary hypertension
J Thorac Cardiovasc Surg
Pulmonary arterial hypertension
Circulation
Right ventricular function and failure: report of a National Heart, Lung, and Blood Institute working group on cellular and molecular mechanisms of right heart failure
Circulation
The role of the right ventricle in pulmonary arterial hypertension
Eur Respir Rev
Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era
Circulation
Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)
Circulation
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry
Ann Intern Med
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2021, JACC: Cardiovascular ImagingCitation Excerpt :Moreover, a disproportionate increase in right ventricular mass index (RVMI) compared with RVEDV indicates concentric hypertrophy and is associated with a favorable outcome in IPAH (13). Eccentric hypertrophy with a disproportionate increase in RVEDV compared with RVMI is considered a maladaptive response to increased afterload and is associated with a poor outcome (13,41). In IPAH, therefore, caution should be exercised when using mass measurements in isolation because they give incomplete information on RV adaptation.