A 29 year old unmarried female presents with progressive dyspnea. Her X ray chest shows clear lung fields. Pulmonary function testing reveals in FVC of92%; FEV1/FVC of 89%; and DLCO of 59%. On exercise testing her oxygen saturation drops from 92% to 86%. What is the likely diagnosis: (AIIMS Nov 2013)
|B||Primary pulmonary hypertension|
|C||Interstitial lung disease|
Primary pulmonary hypertension
1). Fatigue is the most common symptom
3). Syncope and angina, particularly with exertion, are late manifestations of the disease, suggesting the presence of severe pulmonary hypertension causing reduced cardiac output.
4). P2 is loud
5). Narrow splitting of S2
6). Systolic ejection click, the intensity of this click increases on expiration.
FVC is normal, FEV1 is normal & FEV1/FVC is normal.
1). Pulmonary function studies to evaluate for the presence of other causes contributing to the development of pulmonary hypertension.
2). In patients without underlying parenchymal lung disease who have PPH, there have mild restrictive changes but there is no evidence of obstruction.
3). Hypoxemia is a feature and the diffusing capacity for carbon monoxide (DLCO) is reduced.
4). The ECG may indicate right ventricular enlargement or show right ventricular strain.
5). CXR may show enlargement of the pulmonary arteries.
6). An echocardiogram can estimate pulmonary artery pressures, show evidence of tricuspid regurgitation.
Features of Primary Pulmonary Hypertension / Idiopathic Pulmonary Arterial Hypertension
1). Most common in Young women who are 20-40 years of Age (Robbin's 7th/Pg 745) (strong female predominance)
2). Most common symptom is exertional dyspnea
3). Pulmonary Function tests
a. Are usually normal with the exception of DLCO which is characteristically reduced.
b. Non obstructive PFT (FEV1/FVC>0.7 or 70%)
c. Nonrestrictive PFT (FVC > 80%)
4). Exercise tolerance
5). Exercise tolerance is markedly reduced
6). (The exercise capacity of these patients appears to be limited by a low cardiac output because of a decrease in the functional pulmonary vascular bed)