Table 2. Characteristics of the five patients who were not identified according to the screening algorithm.
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | |
|---|---|---|---|---|---|
| Age at CTEPH diagnosis | 76 | 86 | 62 | 65 | 65 |
| Sex | Male | Female | Male | Male | Female |
| NYHA classification at the time of CTEPH referral | 3 | 4 | 3 | 2 | 2 |
| Number of previous VTE events | 2012: provoked PE (postsurgery, malignancy related) | 1994: provoked PE, malignancy related | 1999: unprovoked PE 2014: unprovoked PE |
2002: unprovoked PE 2012: unprovoked DVT |
2014: unprovoked PE |
| Referral to the VUMC (months after PE diagnosis) | 23 | 240 | 6 | 151 | 6 |
| Cardiopulmonary comorbidities | None | COPD | None | None | None |
| Other risk factors for CTEPH a | None | Splenectomy | None | None | Rheumatoid arthritis |
| Clinical prediction score | 2 points | 5 points | 11 points | 9 points | 11 points |
| Persistence of symptoms after index PE | In 2014, new, progressive symptoms of dyspnea | In 2013, new, progressive symptoms of dyspnea | Yes | Yes | Yes |
| Rule-out criteria | Abnormal | Abnormal | Normal | Normal | Normal |
| NT-proBNP ng/L b | 1,694 (<486) | 9,082 (<738) | 101 (<210) | 56 (<376) | 148 (<301) |
| ECG items c | 1 item | 2 items | None | None | None |
| Echocardiography (at diagnosis of CTEPH) | Dilated RV, severe PH | Dilated RV, severe PH | RV not dilated, normal function, signs of PH based on a slightly dilated right atrium and a SPAP of > 44 mm Hg | RV not dilated, normal function, signs of PH based on midsystolic notching of the pulmonary valve and a SPAP of >55 mm Hg | RV not dilated, normal function, no signs of PH. RHC performed because of severity of symptoms and the extensiveness of the abnormalities on V/Q lung scintigraphy |
| RHC mPAP (mm Hg)/PVR (dynes/s/cm 5 ) | 56/554 | 49/577 | 36/329 | 31/400 | 32/376 |
Abbreviations: CTEPH, chronic thromboembolic pulmonary hypertension; ECG, electrocardiography; mPAP, mean pulmonary artery pressure; ng/L, nanograms per liter; NT-proBNP, N-terminal pro-brain natriuretic peptide; NYHA, New York Heart Association; PE, pulmonary embolism; PH, pulmonary hypertension; PM, pacemaker; PVR, pulmonary vascular resistance; RHC, right heart catheterization; RV, right ventricle; SPAP, systolic pulmonary artery pressure; V/Q, ventilation/perfusion lung scintigraphy; VTE, venous thromboembolism; VUMC, VU University Medical Center, Amsterdam.
Splenectomy, infected PM leads, autoimmune diseases.
Age and sex adjusted.
Right bundle branch block: rSR′ or RSr′ pattern in lead V1 with a QRS duration ≥ 120 ms, R:S > 1 in lead V1 with R > 0.5 mV or right QRS axis deviation QRS axis > 90 degrees.