A 27-year-old woman presents with 6 months of progressive exertional dyspnea and near syncope. Vital signs show BP 118/76 mmHg, HR 108 bpm, RR 24/min, SpO2 88% on room air. Examination reveals a loud pulmonic component of S2 and right ventricular heave. Chest X-ray shows normal cardiac silhouette without pulmonary infiltrates. Echocardiography demonstrates elevated pulmonary artery pressure without left-sided heart disease or intracardiac shunting. Which diagnosis best explains these findings?
- A)Primary pulmonary hypertension due to BMPR2 mutationGABARITO
- B)Chronic thromboembolic pulmonary hypertension
- C)Spontaneous pneumothorax
- D)Pulmonary embolism
- E)Pulmonary edema due to left sided heart failure
Explicação
Primary pulmonary hypertension due to BMPR2 mutation is correct. A young woman with progressive dyspnea, loud P2, and isolated pulmonary arterial hypertension without left sided disease fits this classic presentation. The disease involves vascular smooth muscl... Ver explicação completa e trilha adaptativa →