Background: Balloon pulmonary angioplasty (BPA) is a form of therapy for chronic thromboembolic pulmonary hypertension (CTEPH). The study objective is to assess the clinical usefulness of resting ECG (PH-ECG score) in monitoring the efficacy of BPA in CTEPH patients. Methods and results: Ninety-four (n = 94) CTEPH patients were included in the analysis. A standard 12-lead-ECG was performed before the first BPA session and after completion of treatment. The whole analysed population (n = 94) was divided into the following two groups: derivation cohort (n = 41) and validation cohort (n = 53). The derivation cohort was divided into the following two subgroups: patients with mean pulmonary artery pressure (mPAP) after the completion of therapy < 25 mmHg (n = 21) and patients with mPAP after the completion of therapy ≥ 25 mmHg (n = 20). In the first subgroup, four (R-wave V1 + S-wave V5/V6 > 10.5mm, QRS-wave axis > 110degrees, R-wave V1 > S-wave V1, SIQIII pattern) of the six ECG parameters of overload of the right cardiac chambers showed statistically significant differences (p < 0.005). That was followed by a determination of the sensitivity and specificity, positive (PPV) and negative predictive value (NPV), and ROC curve (AUC 0.9; 95% CI: 0.792–1.000) for the variable that was a sum of the above four ECG parameters (PH-ECG score). The absence of all of the four ECG parameters at rest (PH-ECG score = 0) well reflected patients with mPAP < 25 mmHg (sensitivity, 100%; specificity, 80%; PPV, 84%; NPV, 100%). In the validation cohort with mPAP < 25 mmHg and PH-ECG score = 0, sensitivity, specificity, PPV, and NPV were 86%, 77%, 73%, and 89%, respectively. Conclusions: Resting ECG trace is clinically useful in the monitoring of therapeutical effects of BPA in CTEPH patients.