Mandatory medical device diagnostics.
- Echocardiography (echo; cardiac ultrasound) – to measure tricuspid regurgitation (leakage leading to backflow of blood from the right ventricle into the right atrium) and so-called TAPSE (abbreviation for: “tricuspid annular plane systolic excursion”); this allows indirect estimation of systolic pulmonary arterial pressure; measurement of TAPSE is performed using M-mode and describes the longitudinal excursion of the tricuspid valve during systole/contraction phase of the heart (< 2 cm = pulmonary hypertension/pulmonary hypertension). Furthermore, estimation of systolic pulmonary arterial pressure via the maximum regurgitation velocity at the tricuspid valve (TRV; tricuspid valve regurgitation velocity) in continuous Doppler.Objective: to graduate the echocardiographic probability for the presence of pulmonary hypertension (PH) based on the maximum tricuspid valve regurgitation velocity as high, intermediate, or low.
- [Estimated PAPsys:
- 37-50 mmHg (tricuspid regurgitation jet 2.9-3.4 m/sec); PH possible → clarification in the presence of PH risk factors (e.g., scleroderma), right heart strain signs, or unclear dyspnea.
- > 50 mmHg (tricuspid regurgitation jet > 3.4 m/sec): PH likely → workup required, including right heart catheterization (I, C)]
- If PAH (pulmonary arterial hypertension) or CTEPH (chronic thromboembolic pulmonary hypertension) is suspected, and if there is evidence of severe PH associated with left heart or lung disease → presentation of the patient to a specialized PH expert center.
- Electrocardiogram (ECG; recording of the electrical activities of the heart muscle) – Change in ECG usually occurs late or is absent in a large proportion of patients. The following change may occur in cor pulmonale:
- Right heart hypertrophy sign (sign of right heart enlargement):
- Elevation of the R-wave in leads V1 and V2.
- Increase of the S-wave in leads V5 and V6 to > 0, 7 mV.
- Right ventricular repolarization dysfunction:
- ST depressions and T negativities in leads V1-V3.
- Criteria with low specificity (probability that actually healthy individuals who do not have the disease in question are also identified as healthy by the procedure):
- Right bundle branch block with a deformation of the ventricular complex in chest wall leads V1, V2, and a negative T in V1 to V3.
- Pyramidal P pulmonale (P wave is broadened and elevated) in limb lead III
- Right heart hypertrophy sign (sign of right heart enlargement):
- X-ray of the thorax (X-ray thorax/chest), in two planes – changes also show up very late in this case. The following changes may occur in cor pulmonale:
- Right heart hypertrophy, the heart fills the retrosternal space in the lateral image.
- Prominent pulmonary arch (truncus pulmonalis).
- Dilated central pulmonary arteries, caliber jump towards peripheral arteries → peripheral “bright lung“.
- Right heart catheterization (RHC); with the aid of the right heart catheter, right ventricular pressure (pressure in the right ventricle) can be determined at rest and under stress:
- Confirmation of the diagnosis of PH
- Clarification of cause and determination of hemodynamic severity.
- Vasoreactivity testing (with inhaled nitric oxide (NO) or alternatively inhaled iloprost) to identify so-called “responders” who may benefit from therapy with high-dose calcium antagonists; indications: Patients with idiopathic, hereditary, or drug-associated pulmonary arterial hypertension.
Optional medical device diagnostics – depending on the results of the history, physical examination, laboratory diagnostics and obligatory medical device diagnostics – for differential diagnosis.
- Abdominal ultrasonography (ultrasound examination of the abdominal organs) – for basic diagnostics.
- Computed tomography of the thorax/chest (thoracic CT) – for advanced diagnostics.
- Perfusion/ventilation scintigraphy – diagnostic nuclear medicine procedure used to evaluate pulmonary embolism;
- Indications: severe PH; exclusion of chronic thromboembolic pulmonary hypertension.
- Suspected chronic thromboembolic PH (CTEPH); typical in condition n. pulmonary embolism and subsequent exertional dyspnea (shortness of breath on exertion) [method of choice to exclude CTEPH].
- Spiroergometry (measurement of respiratory gases during physical exertion).
- 6-minute walk test – standardized procedure for objective assessment, determination of severity, and progression of exercise limitation attributable to cardiopulmonary causes.