A 46-year-old man with known kyphoscoliosis complains of fatigue and progressive shortness of breath over the last year. He can barely walk a block without becoming "short-winded." During his last evaluation, his vital capacity was 40% of predicted value and his FEV₁/FVC ratio was 85%. He does not use tobacco, alcohol, or illicit drugs.
The patient's blood pressure is 144/78 mm Hg and pulse is 78/min. Oxygen saturation is 90% on room air. The angle of scoliosis is > 65 degrees and kyphosis deformity is above T10. The remainder of physical examination is unremarkable.
Transthoracic echocardiogram shows normal left ventricular function, mild right ventricular enlargement, and estimated pulmonary artery systolic pressure of 55 mm Hg. Pulmonary function test results are unchanged from prior testing.
This patient is most likely to benefit from which of the following?

Question

Quizplus · Accepted Answer

Diseases of the chest wall, such as ankylosing spondylitis, pectus excavatum, and kyphoscoliosis, and morbid obesity cause a restrictive pattern of respiratory impairment that can lead to respiratory failure. Patients with kyphoscoliosis and deformity above T10 are at higher risk for respiratory failure due to compromised chest wall mechanics. pectus excavatum Total lung capacity, vital capacity, and functional residual capacity are reduced due to decreased chest wall compliance. Lung compliance also decreases eventually due to atelectasis and air trapping, causing increased work of breathing. This may lead to respiratory fatigue and even failure. Patients are also prone to nocturnal hypoventilation leading to apneic events. They can subsequently develop pulmonary hypertension and cor pulmonale. Patients at risk for respiratory failure should be advised to avoid extremes of temperature, humidity, fatigue, sedatives, and opiates. They should also receive influenza and pneumococcal vaccines. Treatment of respiratory impairment due to chest wall disease is usually supportive with pulmonary rehabilitation and intermittent nocturnal or longer term use of noninvasive positive-pressure ventilation (NPPV). NPPV eliminates hypoxemia and obstructive apneas and improves lung volumes and respiratory function. Some studies have shown improved mortality in these patients. (Choice A) Bracing may be used in idiopathic scoliosis for skeletally immature adolescents but is contraindicated in adults. (Choice B) Inhaled corticosteroids are not helpful in patients with respiratory compromise from chest wall disease. (Choices D and E) Anticoagulation is recommended for patients with idiopathic or hereditary pulmonary hypertension (PH), drug-induced PH, and chronic thromboembolic PH. PH due to chest wall disorders usually does not benefit from vasodilator or chronic anticoagulation therapy. NPPV is the only therapy proven to improve dyspnea and sleep disturbances and may even partially reverse cor pulmonale. Educational objective: The intermittent use of noninvasive positive-pressure ventilation in patients with chronic respiratory failure from chest wall disease has been shown to improve dyspnea, sleep disturbances, and mortality. It also may partially reverse cor pulmonale. __________ References: Treatment of chronic respiratory failure in kyphoscoliosis: oxygen or ventilation? (http://www.ncbi.nlm.nih.gov/pubmed/14516146) Lung function in congenital kyphosis and kyphoscoliosis. (http://www.ncbi.nlm.nih.gov/pubmed/17473639) Effect of long term intermittent nocturnal non-invasive positive pressure ventilation on patient with severe kyphoscoliosis and hypoxemia. (http://www.ncbi.nlm.nih.gov/pubmed/21686653) Pulmonary diseases and the heart. (http://www.ncbi.nlm.nih.gov/pubmed/18086941)

A 46-Year-Old Man with Known Kyphoscoliosis Complains of Fatigue and Progressive