Pulmonary Hypertension Diagnosis and Risk Assessment Quiz

Study Notes

Pulmonary Hypertension: Diagnosis and Risk Assessment

FPAH can be caused by a mutation in one of the BMPR2 genes inherited from either parent, with a 10% overall risk for family members.
Physical examination may reveal signs such as increased P2 intensity, systolic ejection murmur, right ventricular heave, and jugular venous pulse abnormalities.
Right ventricular failure in PAH patients can lead to systemic venous hypertension and cor pulmonale, with associated signs such as hepatomegaly, ascites, and peripheral edema.
Blood tests, chest X-rays, ECG, echocardiogram, pulmonary function tests, six-minute walk test, nuclear scan, and right-heart catheterization are used for investigations and diagnosis of PH.
The six-minute walk test is used to assess exercise tolerance, with specific threshold values indicating mortality and survival rates.
Right-heart catheterization is the gold standard for diagnosing and classifying PH, providing accurate diagnosis for pulmonary hypertension.
WHO classification and risk assessment tools, such as the 2015 ESC/ERS three-strata risk assessment, are used to evaluate PAH severity and track treatment effectiveness.
Patient-reported outcome measures (PROM) questionnaires, including CAMPHOR, emPHasis-10, and LPHQ, are used to assess the impact of pulmonary hypertension on patients' lives.