Anonymous
06-23-2004, 07:42 PM
A 30-year-old otherwise healthy woman presents to her physician
with complaints of fatigue and dyspnea. Physical examination reveals
normal breath sounds and the presence of third and fourth heart sounds.
Chest x-ray shows clear lung fields but right ventricular enlargement,
main pulmonary artery enlargement, and "pruning" of the peripheral
vasculature. Electrocardiogram shows right axis deviation and right
ventricular hypertrophy. Left ventricular function appears normal
on echocardiography. Serologic studies show antinuclear antibodies.
Which of the following pathological findings would this patient
also show, either at autopsy or if an appropriate biopsy was taken?
A. Mural thrombus of the right atrium
B. Necrosis and scarring of the left ventricle
C. Plexogenic pulmonary vasculopathy
D. Pulmonary artery stenosis
E. Severe pulmonary fibrosis
ans:
The correct answer is C. The presentation described is classic for pulmonary hypertension, and, more specifically, the primary idiopathic form of pulmonary hypertension. This rare condition is suspected of being related to the collagen vascular diseases, since up to 50% of patients have antinuclear antibodies (despite the absence of frank presentation of other autoimmune disease). Also, a similar, known secondary form of pulmonary hypertension is sometimes seen in patients with a wide variety of collagen vascular diseases, including systemic lupus erythematosus, polymyositis, dermatomyositis, systemic sclerosis, and adult and juvenile forms of rheumatoid arthritis. A wide variety of other conditions have also been associated with secondary pulmonary hypertension, including shunts, left atrial hypertension, chronic hypoxia, pulmonary embolism, drug reaction, hepatic cirrhosis, and sickle cell disease. Both primary and secondary forms of pulmonary hypertension are associated with prominent changes in the pulmonary vasculature, which can include muscularization of smaller arterioles, concentric hypertrophy of the intima ("onion skinning"), and a distinctive plexiform lesion (plexogenic pulmonary vasculopathy) in which the smallest arterioles become markedly dilated with lumens partially occluded by endothelial (or possibly mesenchymal) cells and sometimes, thrombus. The prognosis of untreated pulmonary hypertension is poor. However, the use of the vasodilator hydralazine with anticoagulation can slow the course (fatal in about 3 years in untreated patients). If the pulmonary hypertension is secondary, therapy of the primary disease can be helpful.
Unlike cor pulmonale, atrial fibrillation with mural thrombus (choice A) formation is uncommon in primary pulmonary hypertension.
The absence of left ventricular findings on echocardiography tends to exclude myocardial infarction as the source of the patient's findings (choice B).
The presence of enlargement of the main pulmonary artery excludes pulmonary artery stenosis (choice D).
The clear lung fields exclude severe pulmonary fibrosis (choice E).
with complaints of fatigue and dyspnea. Physical examination reveals
normal breath sounds and the presence of third and fourth heart sounds.
Chest x-ray shows clear lung fields but right ventricular enlargement,
main pulmonary artery enlargement, and "pruning" of the peripheral
vasculature. Electrocardiogram shows right axis deviation and right
ventricular hypertrophy. Left ventricular function appears normal
on echocardiography. Serologic studies show antinuclear antibodies.
Which of the following pathological findings would this patient
also show, either at autopsy or if an appropriate biopsy was taken?
A. Mural thrombus of the right atrium
B. Necrosis and scarring of the left ventricle
C. Plexogenic pulmonary vasculopathy
D. Pulmonary artery stenosis
E. Severe pulmonary fibrosis
ans:
The correct answer is C. The presentation described is classic for pulmonary hypertension, and, more specifically, the primary idiopathic form of pulmonary hypertension. This rare condition is suspected of being related to the collagen vascular diseases, since up to 50% of patients have antinuclear antibodies (despite the absence of frank presentation of other autoimmune disease). Also, a similar, known secondary form of pulmonary hypertension is sometimes seen in patients with a wide variety of collagen vascular diseases, including systemic lupus erythematosus, polymyositis, dermatomyositis, systemic sclerosis, and adult and juvenile forms of rheumatoid arthritis. A wide variety of other conditions have also been associated with secondary pulmonary hypertension, including shunts, left atrial hypertension, chronic hypoxia, pulmonary embolism, drug reaction, hepatic cirrhosis, and sickle cell disease. Both primary and secondary forms of pulmonary hypertension are associated with prominent changes in the pulmonary vasculature, which can include muscularization of smaller arterioles, concentric hypertrophy of the intima ("onion skinning"), and a distinctive plexiform lesion (plexogenic pulmonary vasculopathy) in which the smallest arterioles become markedly dilated with lumens partially occluded by endothelial (or possibly mesenchymal) cells and sometimes, thrombus. The prognosis of untreated pulmonary hypertension is poor. However, the use of the vasodilator hydralazine with anticoagulation can slow the course (fatal in about 3 years in untreated patients). If the pulmonary hypertension is secondary, therapy of the primary disease can be helpful.
Unlike cor pulmonale, atrial fibrillation with mural thrombus (choice A) formation is uncommon in primary pulmonary hypertension.
The absence of left ventricular findings on echocardiography tends to exclude myocardial infarction as the source of the patient's findings (choice B).
The presence of enlargement of the main pulmonary artery excludes pulmonary artery stenosis (choice D).
The clear lung fields exclude severe pulmonary fibrosis (choice E).