Practice questions answers, Pulmonary hypertension

Practice questions answers, Pulmonary hypertension

© Jun Wang, MD, PhD

 

1. B. The presentations of exertional dyspnea and dry cough are suggestive of disorders involving heart and/or lung. In a patient with long history of cigarette smoking, with the presence of perioral cyanosis, diminished breath sound and bilateral crackles, it is likely to be a form of chronic obstructive pulmonary disease. The findings of jugular vein distention and lower extremity edema are suggestive of elevated right atrial pressure that may be associated with tricuspid stenosis or pulmonary hypertension. The presence of loud second, in this background, is likely caused by P2, suggestive of pulmonary hypertension. Based on these information, this patient might have group 3 pulmonary hypertension, pulmonary hypertension due to lung diseases and/or hypoxia. In these patient, diagnoses of pulmonary hypertension and underlying lung disorder are needed. Therefore, chest X-ray would be a convenient initial exam to be performed. Arterial blood gas test would provide information for blood oxygen levels and acid-base balance. These results are not quite useful in diagnosing the heart and lung disorder. In this patient, hypoxemia is likely due to presence of cyanosis. So ABG is less likely to be very useful. Complete blood count would not provide enough information for the heart and lung disorders either, even though the white count might be elevated. Similarly, liver function tests are not useful, even though they might be abnormal due to possible liver congestion, secondary to elevated central venous pressure. Troponin levels are elevated due to myocardial injury, that is not very likely in this patient, due to normal vital signs and lack of relevant symptoms.

2. C. X-ray findings of dilated pulmonary trunks and pruning of peripheral pulmonary vessels are consistent with pulmonary hypertension. Echocardiogram should be performed to estimate probability of pulmonary hypertension in pts with associated presentations. The patient may have COPD, but most importantly, he has symptoms and signs of pulmonary hypertension. Blood pH, coronary angiogram, liver and renal function tests, although may have abnormal results, would not contribute to the diagnosis of pulmonary hypertension.

3. B. The diagnostic exam to confirm pulmonary hypertension is right heart catheterization, since it can measure pulmonary artery pressure and estimate pulmonary capillary wedge pressure. Lung biopsy and pulmonary function tests are likely to yield findings for his underlying COPD, but are not helpful in diagnosis of pulmonary hypertension. He has no presentations of pneumonia or pulmonary embolism, based on his normal CBC and D-dimer levels, so sputum culture and V/Q scanning are not going to yield any useful information.

4. D. Echocardiogram finding of dilated right ventricle, with previous findings of loud S2 and dilated pulmonary trunk, is consistent with pulmonary hypertension. He has no presentations of pneumonia or pulmonary embolism, based on his normal CBC and D-dimer levels. Echocardiogram did not find any evidence of septal defect in this case. In addition, he does not have any physical examination findings suggesting congenital ventricular septal defects, such as murmurs. Due to normal vital signs, regular heart rate, and lack of chest pain, etc., myocardial infarct is less likely.

5. C. This patient has a history of heavy cigarette smoking and physical examination reveals perioral cyanosis and bilateral wheezing and crackles. These are consistent with chronic obstructive pulmonary disease. Therefore his pulmonary hypertension is most likely group 3, pulmonary hypertension due to lung diseases and/or hypoxia. Arrythmia and cardiomyopathy may cause group 2 pulmonary hypertension due to left-sided heart disease. Thromboembolism of pulmonary arteries may cause group 4 pulmonary hypertension due to pulmonary artery obstruction. However, there is no evidence for these disorders in this patient. This patient has no presentations of pneumonia or pulmonary embolism, based on his normal CBC and D-dimer levels.

6. C. One of the most important pathological processes for pulmonary hypertension is narrowed pulmonary arteries, due to medial hyperplasia. Irregular glandular growth lined by cells with pleomorphic nuclei is seen in adenocarcinomas, including adenocarcinoma of lung. Lobular neutrophilic infiltration is seen in pneumonia. Microthrombi is seen in pulmonary embolism and some bleeding disorders, including thrombotic thrombocytopenic purpura and disseminated intravascular coagulation, etc. Thickened interalveolar septa with dense fibrosis is seen in interstitial pneumonia.

7. C. Pulmonary hypertension in a patient with COPD, without evidence of left heart disease and thromboembolism, is most likely group 3 pulmonary hypertension, pulmonary hypertension due to lung diseases and/or hypoxia. Group 1, pulmonary arterial hypertension (PAH), is a diagnosis made after ruling out left heart disorder, lung diseases, thromboembolism and other causes of pulmonary hypertension. Group 2 pulmonary hypertension due to left-sided heart disease is associated with left heart disorders, including myocardial infarction, arrythmia, etc., that lead to left ventricular dysfunction. Group 4 pulmonary hypertension due to pulmonary artery obstruction is associated with obstruction of pulmonary arteries, that might be cause by pulmonary thromboembolism, pulmonary artery tumors or stenosis. Group 5 PH, pulmonary hypertension with unclear or multifactorial etiologies, may be associated with hematologic disorders, systemic disorders, end-stage renal disease, or congenital heart diseases, but not chronic lung diseases or hypoxia.

8. C. See discussion of question 2.

9. D. See discussion of question 3. Blood brain natriuretic peptide may be elevated in patients with heart failure, including those with pulmonary hypertension, but the result is not specific nor diagnostic for pulmonary hypertension.

10. A. This is a pulmonary hypertension with history of hypertension, physical and EKG findings suggestive of left heart failure. In these patients, elevated pulmonary artery pressure is a passive response to elevated left atrial pressure. Pulmonary capillary wedge pressure is a relatively accurate estimate of left atrial pressure. It is elevated in these patient with pulmonary hypertension due to left-sided heart disease. Blood brain natriuretic peptide may be elevated, NOT reduced, in patients with heart failure, including those with pulmonary hypertension. Mediastinal lymphadenopathy may be associated with lung inflammation or cancer, but not pulmonary hypertension. Thromboembolism of pulmonary arteries is less likely due to normal coagulation panel result, including a normal D-dimer level. Schistosomiasis infection may be associated with pulmonary arterial hypertension (PAH), a diagnosis made after ruling out left heart disorder, lung diseases, thromboembolism and other causes of pulmonary hypertension.

11. E. Pulmonary hypertension is defined as mPAP > 25 mm Hg. Acute pulmonary embolism is less likely due to normal coagulation panel results. Congenital tricuspid regurgitation usually have presentations starting at young age. Cor pulmonale has presentations of right ventricular hypertrophy, that is not seen in the current case. Coronary heart disease is less likely due to lack of associated symptoms, such as chest pain, etc.

12. C. Elevated pulmonary capillary wedge pressure is consistent with elevated left atrial filling pressure, commonly associated with left ventricular dysfunction. COPD is associated with group 3 pulmonary hypertension, pulmonary hypertension due to lung diseases and/or hypoxia. Congenital tricuspid stenosis is associated with right atrial dilation and hypertrophy, but usually do not cause left atrial pressure elevation. Pulmonary artery malformation may cause precapillary pulmonary hypertension, without elevation of pulmonary capillary wedge pressure. Recurrent pulmonary embolism can cause group 4 pulmonary hypertension due to pulmonary artery obstruction due to obstruction of pulmonary arteries. It is a precapillary pulmonary hypertension, without elevation of pulmonary capillary wedge pressure.

13. A. See discussion of question 10. Pulmonary arteriole intima hyperplasia may cause precapillary pulmonary hypertension, without elevation of pulmonary capillary wedge pressure. It may be seen in pulmonary arterial hypertension (PAH), pulmonary hypertension due to lung diseases and/or hypoxia and pulmonary hypertension due to pulmonary artery obstruction. Pulmonary thromboembolism and pulmonary valve stenosis are associated with pulmonary hypertension due to pulmonary artery obstruction. Right heart dysfunction may cause elevated jugular vein pressure, but not pulmonary hypertension. It can be resulted from pulmonary hypertension, as seen in cor pulmonale.

14. E. Although the clinical presentations are not specific, the physical examination findings of loud S2, in a patient with normal blood pressure, is suggestive of pulmonary hypertension. What unique in this patient is the history of deep vein thrombosis, with the physical finding of irregularly irregular heart rate and flow murmurs over lung fields, are indicative for hyper-coagulable state and subsequent thromboembolism, including pulmonary thromboembolism. Therefore, V/Q scan is a needed to evaluate the possibility of pulmonary embolism. Chest X-ray findings may be consistent with pulmonary hypertension, but would not be contributory to identify the underlying causes. The patient does not have clinical presentation of myocardial ischemia, so coronary angiogram and EGK would not be very useful, even though certain degrees of abnormalities might be revealed, due to the history of cardiomyopathy. Thyroid sonography is irrelevant, since Hashimoto thyroiditis usually causes hypothyroidism, but has no known connection to pulmonary hypertension.

15. B. In a patient with V/Q scan result suggestive of pulmonary embolism, angiogram is used to confirm the diagnosis. CT-guided biopsy is commonly used for diagnosis of neoplasms of lung, but not for pulmonary embolism. Magnetic resonance pulmonary angiography may be used in diagnosis of PE in patients when CTPA or V/Q scan cannot be performed, but not a first-line diagnostic test. D-dimer will be elevated in patients with thrombosis at any location, it is not specific for lung thromboembolism. Thyroid biopsy may reveal findings of Hashimoto thyroiditis. It usually causes hypothyroidism, but has no known connection to pulmonary hypertension.

16. D. See discussion of questions 3 and 15. The purpose for diagnosing pulmonary hypertension is to confirm elevated pulmonary artery pressure, as well as identify underlying causes. For this patient, the presence of pulmonary thromboembolism is confirmed by V/Q scan and pulmonary angiogram, right heart catheterization is needed to confirm pulmonary hypertension (Maron et al., 2021).

17. C. D-dimer will be elevated in patients with thrombosis at any location, including lung thromboembolism. Fibrinogen level will be reduced in these patients. Eosinophil count is elevated commonly due to allergy, parasitic infection, or neoplastic process. Platelet count may be reduced or elevated, or normal in patients with clinical presentations of thrombosis, depending on underlying causes. For example, it may be elevated in myeloproliferative neoplasms, with clinical presentations of thrombosis and/or bleeding, or reduced in heparin induced thrombocytopenia, with presentations of thrombosis. Elevated pulmonary capillary wedge pressure is seen in patients with elevated left atrial filling pressure, commonly associated with left ventricular dysfunction. Troponin is elevated in myocardial infarction. This patient does not have clinical presentations of these disorders.

18. D. Pulmonary hypertension is defined as mPAP > 25 mm Hg. This is a case is a group 4 pulmonary hypertension due to pulmonary artery obstruction, based on evidence of pulmonary artery thromboembolism and pulmonary hypertension. Bronchopneumonia usually do not cause elevated pulmonary artery pressure. It is less likely in this patient due to lack of fever. Cor pulmonale has presentations of right ventricular hypertrophy, that is not seen in the current case. Left heart dysfunction may cause group 2 pulmonary hypertension due to left-sided heart disease with an elevated pulmonary capillary wedge pressure. Superior vena cava syndrome is associated with obstruction of superior vena cava, that results in reduced filling of right atrium and ventricle. It may cause obstructive shock, but not pulmonary hypertension.

19. C. The key pathological change in group 4 pulmonary hypertension due to pulmonary artery obstruction, due to chronic pulmonary thromboembolism, is the presence of unsolved thromboemboli in pulmonary arteries. These thromboemboli are incorporated into vascular wall through fibrothrombotic organization, that will lead to vascular occlusion and remodeling. Congenital pulmonary artery stenosis may cause group 4 pulmonary hypertension due to pulmonary artery obstruction, but usually do not have presentations of thromboembolism. Left ventricular hypertrophy may be seen in patients with left ventricular dysfunction that may be associated with group 2 pulmonary hypertension due to left-sided heart disease. Theses patients have an elevated pulmonary capillary wedge pressure. Pulmonary interstitial fibrosis may be associated with pulmonary hypertension, pulmonary hypertension due to lung diseases and/or hypoxia. However, patients with interstitial pulmonary fibrosis usually have fine end-inspiratory crackles, digital clubbing, besides presentations of pulmonary hypertension. These findings are not seen in this patient. Singular millimetric fibrovascular lesions are commonly seen in pulmonary arterial hypertension (PAH), especially the hereditary form, but not other groups of pulmonary hypertension.

20. C. See discussion of question 2.

21. E. See discussion of question 3.

22. B. This patient has pulmonary hypertension, but does not have presentations of left heart disease, lung disorders, hypoxia, thromboembolism, murmurs for pulmonary artery stenosis, or other disorders that may cause pulmonary hypertension. Therefore, this is most likely a case of pulmonary arterial hypertension (PAH).

23. C. Although most cases of pulmonary arterial hypertension (PAH) in the US are idiopathic, this patient is unique for his immigration history from a schistosomiasis endemic area. Since schistosomiasis is one of the most common causes of pulmonary arterial hypertension (PAH), fecal parasite studies are needed to detect potential infection. Arterial blood gas test would provide information for blood oxygen levels and acid-base balance. It is not useful in diagnostic workups for pulmonary hypertension.  Chest MRI may detect pulmonary disorders. Pulmonary angiogram is used to confirm pulmonary embolism. Serum troponin is elevated in patients with myocardial injury. This patient does not presentations of lung or heart diseases.

24. C. The pathological changes seen in patients with pulmonary arterial hypertension include proliferative vasculopathy, hyperplasia/hypertrophy of all three layers of vascular wall (intima, media, adventitia), fibrosis, thrombi and SiMFis lesion (singular millimetric fibrovascular lesions). These changes involve small muscular arteries. Alveolar neutrophilic infiltration is seen in active inflammation, such as bronchopneumonia. Left ventricular hypertrophy may be seen in patients with left ventricular dysfunction that may be associated with group 2 pulmonary hypertension due to left-sided heart disease. These patients usually have history of hypertension or aortic stenosis, etc. Organized thrombi are seen in group 4 pulmonary hypertension due to pulmonary artery thromboembolism. Pulmonary interstitial fibrosis may be associated with pulmonary hypertension, pulmonary hypertension due to lung diseases and/or hypoxia. However, patients with interstitial pulmonary fibrosis usually have fine end-inspiratory crackles, digital clubbing, besides presentations of pulmonary hypertension. These findings are not seen in this patient.

25. D. This patient has a history of pulmonary embolism. With presentations of pulmonary hypertension and right heart failure, and an elevated D-dimer, she is most likely having cor pulmonale associated with group 4 pulmonary hypertension due to pulmonary artery obstruction. Pulmonary angiogram is used to confirm pulmonary embolism. Chest MRI may detect pulmonary disorders. CT-guided biopsy is commonly used for diagnosis of neoplasms of lung, but not for pulmonary embolism. Echocardiogram should be performed to estimate probability of pulmonary hypertension in pts with associated presentations. Right heart catheterization is used to confirm pulmonary hypertension. But they usually could not identify the underlying lung disorders.

26. C. This patient has a history of pulmonary embolism. With presentations of pulmonary hypertension and right heart failure, and an elevated D-dimer, she is most likely having cor pulmonale associated with group 4 pulmonary hypertension due to pulmonary artery obstruction. Cor pulmonale is defined as right heart dysfunction associated with lung diseases, commonly due to pulmonary hypertension. Cardiomyopathy, congenital tricuspid regurgitation, and pulmonary valve stenosis may cause right heart failure, but they are not associated with lung disorders. Pulmonary arterial hypertension (PAH) may cause cor pulmonale. In a patient already have presentations of right heart failure, in a background of pulmonary hypertension, the best diagnosis is cor pulmonale. In addition, pulmonary arterial hypertension is a diagnosis made after ruling out left heart disorder, lung diseases, thromboembolism and other causes of pulmonary hypertension. This patient is most likely a group 4 pulmonary hypertension due to pulmonary artery obstruction, associated with pulmonary thromboembolism.

27. E. The key pathological change in group 4 pulmonary hypertension due to pulmonary artery thromboembolism is the presence of unsolved thromboemboli in pulmonary arteries. These thromboemboli are incorporated into vascular wall through fibrothrombotic organization, that will lead to vascular occlusion and remodeling. Usually this is a chronic process involving recurrent pulmonary thromboembolism. Bacterial infection may cause pneumonia, but usually not pulmonary hypertension. Chronic lung diseases and left ventricular dysfunction may be associated with pulmonary hypertension due to lung diseases and/or hypoxia and pulmonary hypertension due to left-sided heart disease. Pulmonary valve stenosis may cause right heart failure. Usually, these are not associated with pulmonary hypertension due to pulmonary artery obstruction.

28. C. This patient has signs of pulmonary hypertension. Since she does not have presentations of lung or heart diseases, or thromboembolism, this is most likely pulmonary arterial hypertension. In addition, her positive family history makes this most likely a hereditary form. This is not a cor pulmonale due to lack of evidence for right heart failure.

29. A. BMPR2 and ALK2 mutations are commonly seen in hereditary pulmonary arterial hypertension. Factor V mutation is associated with hypercoagulable status. PRKAR1A mutation is seen Carney syndrome, that may present as cardiac myxomas. TSC1 mutation is seen in tuberous sclerosis, that may present with rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, renal cell carcinoma, etc.. VHL mutation is seen in von Hippel-Lindau syndrome, that may present with renal cell carcinoma, hemangioblastoma, etc..

 

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