A 29 year old anxious lady presents with a history of progressive breathlessness and exercise intolerance since four months. Her FVC is 90% and FEV I / FVC is 86%. Oxygen saturation after exercise was observed to drop from 92% to 86%. What is the likely diagnosis :

Correct Answer: Primary pulmonary hypeension
Description: Answer is B (Primary pulmonary hypeension): Reduced exercise tolerance and drop in oxygen saturation from 92% to 86% on exercise with a normal FVC and FEVI/FVC is consistent with a diagnosis of Primary Pulmonary Hypeension. Primary Pulmonary Hypeension characteristically presents with exeional dyspnea, exercise intolerance and a normal FVC; FEVI /FVC ratio The most common symptom attributable to pulmonary hypeension is exeional dyspnea and reduced exercise capacity -- Harrison Primary pulmonary hypeension is neither a restrictive nor obstructive pulmonary disease, and hence spirometry results are essentially normal with both FVC and FEVI / FVC ratio > 80% of predicted. Obstructive disease Restrictive disease FEV 1 / FVC is characteristically < 80% of predicted FVC is characteristically < 80% of predicted Interstitial lung disease does not present with a normal FVC Interstitial lung disease should present with a restrictive pattern on 'pulmonary function tests' with a reduced FVC to values less than 80% of predicted. A forced vital capacity of 90% (FVC = 90%) is normal and hence makes a diagnosis of interstitial lung disease unlikely Primary Alveolar Hvpoventilation does not present with dyspnea and exercise intolerance Patients with primary pulmonary hypoventilation may have normal pulmonary function test with a normal FEV1 and normal FVC/FEV 1. However, dyspnea or shoness of breath is remarkably absent or extremely uncommon despite severe aerial blood gas derangements presumably because of impaired chemoreceptor and ventilatory drive. Also exacerbation of hypoxemia or drop in oxygen saturation is characteristically seen during sleep and not on exercise. `Despite severe aerial blood gas derangements is uncommon' - Harrison `Dyspnea is remarkably absent' - Spiral Manual of pulmonary Medicine (Lippincott) 6th/444 Anxiety disorder: Hyperventilation does not present with exercise intolerance Patients with Anxiety associated Hyperventilation may also present with normal spirometry results with a normal FVC and FEV 1 /FVC. Dyspnea may also be a presenting feature; however such patients do not show features of exercise intolerance. 'Patients with psychogenic hyperventilation typically complain of dyspnea at rest, but not during mild exercise. During mild or moderate exercise their hyperventilation tends to disappear' - Harrison
Category: Medicine
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