Signs of right ventricular deterioration in clinically stable patients with pulmonary arterial hypertension.

Chest

PubMedID: 25376008

van de Veerdonk MC, Marcus JT, Westerhof N, de Man FS, Boonstra A, Heymans MW, Bogaard HJ, Vonk-Noordegraaf A. Signs of right ventricular deterioration in clinically stable patients with pulmonary arterial hypertension. Chest. 2015;147(4):1063-71.
Background.
Even after years of stable response to therapy, patients with idiopathic pulmonary arterial hypertension (IPAH) may show an unexpected clinical deterioration due to progressive right ventricular (RV) failure. Therefore, the aim of this study was to assess in five-year clinically stable patients with IPAH whether initial differences or subsequent changes in RV volumes precede late clinical progression.

Methods.
Included were 22 clinically stable patients, reflected by stable or improving NYHA-class II-III and exercise capacity during five years of follow-up. Twelve patients remained subsequently stable during a total follow-up of ten years, while ten other patients showed late progression leading to death or lung-transplantation after a follow-up of eight years. All patients underwent right heart catheterization and cardiac MRI at baseline, 1.5, 3.5, 6.5 and, when still alive, ten years of follow-up.

Results.
Baseline hemodynamics were comparable in both groups and remained unchanged during the entire follow-up period. Baseline RV end-systolic volume (RVESV) was higher and RV ejection fraction (RVEF) was lower in late-progressive patients. Late-progressive patients demonstrated a gradually increased RV end-diastolic volume (RVEDV) and RVESV and, a declined RVEF whereas long-term stable patients did not show any RV changes.

Conclusions.
In five-years stable IPAH patients, subsequent late disease progression is preceded by changes in RV volumes. Our results implicate that monitoring of RV volumes allows the anticipation to clinical worsening, even at the time of apparent clinical stability.