| First author [ref.] | Patients | Therapy | NYHA/WHO FC at initiation of therapy I/II/II/IV % | Treatment length | Main right ventricle-related findings and associations |
| Prospective, multicentre studies | |||||
| Echocardiography | |||||
| Keogh [60] | PAH (various); n=112 | Combination therapy (ERAs/sildenafil/iloprost) | 0/13/61/26 | Mean 7.9±5.6 months | Improvements in right ventricular systolic function and severity of tricuspid regurgitation on sequential measurement |
| Sohn [61] | PAH (various); n=12 | Bosentan | 0/0/100/0 | 12 weeks | Trend 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=16 | Bosentan | 0/0/94/6 | 16 weeks | Significant 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 | |||||
| Echocardiography | |||||
| Duffels [63] | PAH-CHD; n=9 CTEPH; n=9 | Bosentan | 0/11/89/0 | 1 yr | Responders (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=39 | Epoprostenol | 0/0/69/31 | Mean 27±21 months | No significant improvement in right ventricular hypertrophy or strain Improvements in right ventricular function (semi-quantitative assessment) in some patients |
| Ruiz [65] | Severe PAH; n=20 | Sildenafil rescue therapy | Significant reduction of right ventricular end-diastolic diameter and left ventricular diastolic eccentricity index with treatment | ||
| Dyer [66] | Children, IPAH; n=12 | Bosentan | Not given | Median 9 (3–19) months | Tei index decreased significantly in responders to therapy |
| Allanore [67] | SSc with normal Ppa; n=18 | Bosentan | Not given | 4 weeks | Significant improvement in peak systolic and diastolic strain rate |
| Seyfarth [68] | Progressive PAH despite prostanoid therapy; n=16 | Bosentan rescue therapy | 0/37/50/13 | Mean 13.5±5.0 months | Tei index improved from baseline at 6 months and last follow-up |
| Sebbag [69] | IPAH; n=16 | Epoprostenol | 0/0/37.5/62.5 | Mean 5.9±4.6 months | Significant 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 |
| CMRI | |||||
| Chin [70] | PAH (unspecified); n=16 | Bosentan | 12 months | Trend 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=15 | Bosentan/sildenafil combination therapy | 0/13/87/0 | 12 months bosentan mono- therapy followed by 3 months combination therapy | Significant decrease in right ventricular mass and RVEF Improvement in right ventricular end diastolic volume |
| Allanore [67] | SSc with normal Ppa; n=18 | Bosentan | Not given | 4 weeks | Significant improvement in myocardial perfusion index |
| Wilkins [56] | PAH (various); n=26 | Bosentan alone (n=13) or in combination with sildenafil (n=13) | 0/0/100/0 | 16 weeks | Reduction 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=11 | Epoprostenol | 0/0/64/36 | 1 yr | Significant 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 | |||||
| Echocardiography | |||||
| Basil [73] | PAH; n=17 | Not given | Not given | 6 months | Serial improvements in TAPSE are paralleled by improving 6MWD and falling BNP levels |
| Nath [74] | PAH; n=20 | Epoprostenol | Mean 22.7±9.3 months | Peak TRV and Ppa/PVVTI were significantly improved during treatment No significant change in Tei index | |
| CMRI | |||||
| Blalock [75] | PAH (predominantly IPAH); n=12 | Ambrisentan | 0/0/100/0 | 1 and 2 yrs | No 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 MRI | Sildenafil | Not available | 3 months | Reduction 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.