Table 4. Right ventricular response to therapy: descriptive studies
First author [ref.]PatientsTherapyNYHA/WHO FC at initiation of therapy I/II/II/IV %Treatment lengthMain right ventricle-related findings and associations
Prospective, multicentre studies
        Keogh [60]PAH (various); n=112Combination therapy (ERAs/sildenafil/iloprost)0/13/61/26Mean 7.9±5.6 monthsImprovements in right ventricular systolic function and severity of tricuspid regurgitation on sequential measurement
        Sohn [61]PAH (various); n=12Bosentan0/0/100/012 weeksTrend towards decrease in right ventricular end-systolic and end-diastolic areas, and in cardiac index
Significant reduction in maximum TRV
        Sitbon [62]Severe PAH associated with HIV infection; n=16Bosentan0/0/94/616 weeksSignificant decrease in right ventricular area, right ventricular end systolic area, AcT/RVET and end systolic eccentricity index
Improvements in Tei index
Prospective, single-centre studies
        Duffels [63]PAH-CHD; n=9 CTEPH; n=9Bosentan0/11/89/01 yrResponders (n=12) showed significant improvements in right ventricular end diastolic area index, and TVI RVOT index, and improvements in right ventricular early diastolic filling, right ventricular contraction time and Tei index
        Lammers [64]Children; n=39Epoprostenol0/0/69/31Mean 27±21 monthsNo significant improvement in right ventricular hypertrophy or strain
Improvements in right ventricular function (semi-quantitative assessment) in some patients
        Ruiz [65]Severe PAH; n=20Sildenafil rescue therapySignificant reduction of right ventricular end-diastolic diameter and left ventricular diastolic eccentricity index with treatment
        Dyer [66]Children, IPAH; n=12BosentanNot givenMedian 9 (3–19) monthsTei index decreased significantly in responders to therapy
        Allanore [67]SSc with normal Ppa; n=18BosentanNot given4 weeksSignificant improvement in peak systolic and diastolic strain rate
        Seyfarth [68]Progressive PAH despite prostanoid therapy; n=16Bosentan rescue therapy0/37/50/13Mean 13.5±5.0 monthsTei index improved from baseline at 6 months and last follow-up
        Sebbag [69]IPAH; n=16Epoprostenol0/0/37.5/62.5Mean 5.9±4.6 monthsSignificant improvement in Tei index
No significant change in right ventricular size, TRV, cardiac output or heart rate
Improvements in Tei index related to clinical improvements
        Chin [70]PAH (unspecified); n=16Bosentan12 monthsTrend toward improvement in right ventricular stroke, but no change in RVEF or right ventricular end-diastolic volume following treatment
6MWD improved more in patients in whom RVEF increased
Patients who died during follow-up had significantly lower RVEF and left ventricular end-diastolic volume indexes than surviving patients
        van Wolferen [71]PAH; n=15Bosentan/sildenafil combination therapy0/13/87/012 months bosentan mono- therapy followed by 3 months combination therapySignificant decrease in right ventricular mass and RVEF
Improvement in right ventricular end diastolic volume
        Allanore [67]SSc with normal Ppa; n=18BosentanNot given4 weeksSignificant improvement in myocardial perfusion index
        Wilkins [56]PAH (various); n=26Bosentan alone (n=13) or in combination with sildenafil (n=13)0/0/100/016 weeksReduction in right ventricular mass from baseline in patients who completed combination therapy regimen
Significant increase in cardiac index associated with significant reduction in eccentricity index during systole in patients treated with combination therapy
No change in Tei index
        Roeleveld [72]IPAH; n=11Epoprostenol0/0/64/361 yrSignificant improvement in stroke volume associated with significant improvement in cardiac output measured by CMRI
Right ventricular dilatation and hypertrophy were not reversed by epoprostenol therapy, but did not progress
Retrospective, single-centre studies
        Basil [73]PAH; n=17Not givenNot given6 monthsSerial improvements in TAPSE are paralleled by improving 6MWD and falling BNP levels
        Nath [74]PAH; n=20EpoprostenolMean 22.7±9.3 monthsPeak TRV and Ppa/PVVTI were significantly improved during treatment
No significant change in Tei index
        Blalock [75]PAH (predominantly IPAH); n=12Ambrisentan0/0/100/01 and 2 yrsNo significant change from baseline in right and left ventricular volumes and function after 1 yr
Statistically significant improvement in RVEF at 2 yrs compared with baseline, but no significant change in the indexed right ventricular mass or end-diastolic volume
    Michelakis [76]PAH; n=5; n=3 underwent MRISildenafilNot available3 monthsReduction in right ventricular mass
RVEF improved and abnormal septal shift to left ventricle was reversed
  • NYHA/WHO FC: New York Heart Association/World Health Organization functional class; MRI: magnetic resonance imaging; CMRI: cardiac MRI; PAH: pulmonary arterial hypertension; ERA: endothelin receptor antagonist; TRV: tricuspid regurgitant velocity; AcT: acceleration time; RVET: right ventricular ejection time; PAH-CHD: PAH associated with congenital heart disease; CTEPH: chronic thromboembolic pulmonary hypertension; TVI RVOT: tricuspid regurgitant velocity right ventricular outflow time-velocity integral; IPAH: idiopathic PAH; SSc: systemic sclerosis; Ppa: pulmonary artery pressure; RVEF: right ventricular ejection fraction; 6MWD: 6-min walk distance; TAPSE: tricuspid annular plane systolic excursion; BNP: brain natriuretic protein; PVVTI: pulmonary valve velocity time integral.