Echocardiographic and hemodynamic studies have recently been conducted to assess the RV function in idiopathic pulmonary fibrosis [26]. In a retrospective cohort study of 135 IPF patients (FVC 51 % of predicted and DLCO 39 % of predicted), the RVSP for the cohort was 47 ± 18 mmHg and the CI 2.9 ± 0.5; 13 % of the patients had a moderately or severely dilated right atrium and 11 % a moderate to severe degree of RV dysfunction. Patients with a TAPSE < 1.6 cm had a worse survival (Fig. 18.4). The authors concluded that right-sided heart dysfunction was more strongly associated with outcome than the Pa pressure [26]. A study of 25 IFP patients with associated PAH from the Cleveland clinic concluded that noninvasive tests such as echocardiography and the 6 MW distance performed poorly in detecting PAH in IPF patients. Right heart catheterization revealed that their patients had a mean Pap of 33 ± 8 mmHg and, unfortunately, parameters of right or left heart function were not evaluated [27].

Little information of cardiac MRI studies in patients with pulmonary fibrosis exists, with the exception of studies that have examined patients with systemic sclerosis [28–33] many of which have a pulmonary fibrosis disease component. In scleroderma patients with and without interstitial lung disease, with and without pulmonary vascular involvement, fibrosis and microvessel disease of the heart can be present. In patients with sarcoidosis pulmonary vascular disease and pulmonary hypertension can affect the performance of the RV and cardiac MRI is useful to assess or rule out cardiac sarcoidosis as another cause of RVF [34].

The recently described entity of upper lobe emphysema, lower lobe fibrosis, hypoxemia, dyspnea, and highly prevalent pulmonary hypertension (“The syndrome of combined pulmonary fibrosis and emphysema”; [35–37]) is receiving more and more attention because of its poor prognosis. A low value for TAPSE of 11 mm has been reported in a 76-year-old man who hemodynamically improved after treatment with an endothelin receptor blocker plus a PDE 5 inhibitor [38]; a systematic evaluation of RV function in these patients is still lacking.

Right ventricular function in patients with cystic fibrosis with and without PAH has been examined repeatedly by measuring RVEF [38, 39] or echocardiography variables [40, 41] The consensus of these studies appears to be that there is an impairment of RV function (a decreased RVEF) in patients with significant PAH; a recent study from Spain of 37 cystic fibrosis patients concluded that RV functional abnormalities may already be present at early stages of the disease [41], a similar conclusion as the one reached by Hilde et al. [11] after investigating RF function in COPD patients without resting PAH.

Many clinicians over the years had conceptual difficulties to explain for themselves and to their students “cor pulmonale” as a consequence of pulmonary hypertension alone [9], in part because of the frequently acknowledged associated LV dysfunction. COPD, cystic fibrosis, and interstitial fibrosis have as one common denominator lung inflammation and a “sick lung circulation,” thus an inflammation concept of “cor pulmonale” appears now more attractive than an RV afterload hypothesis. An inflammation-related hypothesis [42] of RV dysfunction could even accommodate the results of a single center study of 51 children with asthma; these investigators used tissue Doppler echocardiography to demonstrate subclinical RV dysfunction [43]. Lastly, we would predict that radiation therapy, resulting in lung and heart tissue irradiation [44, 45] causing profound oxidative stress and inflammation (pneumonitis and myocardial injury) would impact not only LV but also RV function.

Every so often the question is raised whether patients with cor pulmonale should receive therapy that targets pulmonary hypertension [46–49]. The opinions vary from nihilism to individualized treatment strategies. The late David Flenley had famously quipped: “patients with COPD die with cor pulmonale, not from it.” A recent meta-analysis of COPD patients treated with pulmonary hypertension targeting therapy concluded that pulmonary hypertension directed treatments have a significant effect in improving exercise capacity in COPD patients with pulmonary hypertension [50]. Yet whether or not pulmonary hypertension targeting therapy is efficacious in patients with COPD remains controversial [51, 52] and the therapy of the underlying disease is still the only recommended option as outlined in the management guidelines of COPD [53]; generally evidence of improved RV function after vasodilator therapy has been lacking.