Practice questions Pulmonary hypertension

Practice questions, Pulmonary hypertension

© Jun Wang, MD, PhD
 
1. Use this case for next 7 questions. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. What initial exam should be performed?
A. Arterial blood gas test
B. Chest X-ray
C. Complete blood count
D. Liver function tests
E. Troponin levels

2. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. Chest X-ray reveals bilateral flattened diaphragm and widened intercostal spaces. No infiltrate nor tumor is seen. The pulmonary arteries are dilated at their trunks, with reduced vascular shadow at periphery. Arterial blood gas test reveals a PaO₂ of 61 mm Hg (normal range: 80-110 mm Hg). His CBC, PT, aPTT and D-dimer are all within normal ranges. What additional abnormal diagnostic test results is likely to establish the diagnosis?
A. Blood pH
B. Coronary angiogram
C. Echocardiogram
D. Liver function tests
E. Renal function tests
 
3. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. Chest X-ray reveals bilateral flattened diaphragm and widened intercostal spaces. No infiltrate nor tumor is seen. The pulmonary arteries are dilated at their trunks, with reduced vascular shadow at periphery. Arterial blood gas test reveals a PaO₂ of 61 mm Hg (normal range: 80-110 mm Hg). His CBC, PT, aPTT and D-dimer are all within normal ranges.
 
Echocardiogram reveals right ventricular dilation. No significant abnormalities are seen in left heart. What exam is likely to confirm the diagnosis?
A. Lung biopsy
B. Right heart catheterization
C. Pulmonary function tests
D. Sputum culture
E. V/Q scan
 
4. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. Chest X-ray reveals bilateral flattened diaphragm and widened intercostal spaces. No infiltrate nor tumor is seen. The pulmonary arteries are dilated at their trunks, with reduced vascular shadow at periphery. Arterial blood gas test reveals a PaO₂ of 61 mm Hg (normal range: 80-110 mm Hg). His CBC, PT, aPTT and D-dimer are all within normal ranges.
 
Echocardiogram reveals right ventricular dilation. No significant abnormalities are seen in left heart. What is most likely the diagnosis?
A. Bronchopneumonia
B. Congenital ventricular septal defect
C. Myocardial infarction
D. Pulmonary hypertension
E. Pulmonary embolism
 
5. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. Chest X-ray reveals bilateral flattened diaphragm and widened intercostal spaces. No infiltrate nor tumor is seen. The pulmonary arteries are dilated at their trunks, with reduced vascular shadow at periphery. Arterial blood gas test reveals a PaO₂ of 61 mm Hg (normal range: 80-110 mm Hg). His CBC, PT, aPTT and D-dimer are all within normal ranges.
 
Echocardiogram reveals right ventricular dilation. No significant abnormalities are seen in left heart. What is most likely causing his presentations?
A. Arrhythmia
B. Cardiomyopathy
C. Chronic pulmonary hypoxia or inflammation
D. Thromboembolism
E. Viral infection
 
6. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. Chest X-ray reveals bilateral flattened diaphragm and widened intercostal spaces. No infiltrate nor tumor is seen. The pulmonary arteries are dilated at their trunks, with reduced vascular shadow at periphery. Arterial blood gas test reveals a PaO₂ of 61 mm Hg (normal range: 80-110 mm Hg). His CBC, PT, aPTT and D-dimer are all within normal ranges.
 
Echocardiogram reveals right ventricular dilation. No significant abnormalities are seen in left heart. What is most likely seen in his lung biopsy?
A. Irregular glandular growth lined by cells with pleomorphic nuclei
B. Lobular neutrophilic infiltration
C. Medial hyperplasia
D. Microthrombi
E. Thickened interalveolar septa with dense fibrosis
 
7. A 62-year-old man presents with dyspnea after activity and dry coughing for 3 months. He does have progressive dyspnea for 6 years. He has a history of type 2 diabetes for 15 years. He has been smoking 2 packs of cigarette per day for the last 40 years. His family history is unremarkable. His vital signs are within normal range. Physical examination reveals perioral cyanosis. Dilated jugular veins are noted. Chest auscultation reveals diminished breath sounds with wheezing crackles bilaterally. He has a rhythmical heart rate with a loud second sound. No significant abnormalities are noted in his abdomen. His lower extremities have edema below the knee. Chest X-ray reveals bilateral flattened diaphragm and widened intercostal spaces. No infiltrate nor tumor is seen. The pulmonary arteries are dilated at their trunks, with reduced vascular shadow at periphery. Arterial blood gas test reveals a PaO₂ of 61 mm Hg (normal range: 80-110 mm Hg). His CBC, PT, aPTT and D-dimer are all within normal ranges. Echocardiogram reveals right ventricular dilation. No significant abnormalities are seen in left heart. What is the correct classification of his disorder?
A. Group 1
B. Group 2
C. Group 3
D. Group 4
E. Group 5
 
 
8. Use this case for next 6 questions. A 52-year-old woman presents with progressive dyspnea for 2 months. She does not have cough, hemoptysis, fever, chest pain or weight loss. She has a history of hypertension for 20 years. Her family history is positive for a few members with hypertension. She does not drink alcohol nor smoke cigarette. She has blood pressure of 152/95 mm Hg. Her other vital signs are within normal range. Physical examination reveals bilateral lower leg edema. She has a loud second heart sound, but no heart murmurs are heard. Pulmonary auscultation reveals rales in both lung bases. No hepatomegaly is noted. Her CBC, liver and renal function tests, and coagulation panels are within normal range. EKG reveals left ventricular hypertrophy. What exam should be performed next?
A. Chest CT
B. Coronary angiogram
C. Echocardiogram
D. Serum troponin levels
E. V/Q scan
 
9. A 52-year-old woman presents with progressive dyspnea for 2 months. She does not have cough, hemoptysis, fever, chest pain or weight loss. She has a history of hypertension for 20 years. Her family history is positive for a few members with hypertension. She does not drink alcohol nor smoke cigarette. She has blood pressure of 152/95 mm Hg. Her other vital signs are within normal range. Physical examination reveals bilateral lower leg edema. She has a loud second heart sound, but no heart murmurs are heard. Pulmonary auscultation reveals rales in both lung bases. No hepatomegaly is noted. Her CBC, liver and renal function tests, and coagulation panels are within normal range. EKG reveals left ventricular hypertrophy.
 
Echocardiogram reveals a peak tricuspid regurgitation velocity of 3.1 m/s (normal < 2.8 m/s). What exam should be performed next?
A. Blood brain natriuretic peptide
B. Coronary angiogram
C. Lung biopsy
D. Right heart catheterization
E. V/Q scan
 
10. A 52-year-old woman presents with progressive dyspnea for 2 months. She does not have cough, hemoptysis, fever, chest pain or weight loss. She has a history of hypertension for 20 years. Her family history is positive for a few members with hypertension. She does not drink alcohol nor smoke cigarette. She has blood pressure of 152/95 mm Hg. Her other vital signs are within normal range. Physical examination reveals bilateral lower leg edema. She has a loud second heart sound, but no heart murmurs are heard. Pulmonary auscultation reveals rales in both lung bases. No hepatomegaly is noted. Her CBC, liver and renal function tests, and coagulation panels are within normal range. EKG reveals left ventricular hypertrophy. Echocardiogram reveals a peak tricuspid regurgitation velocity of 3.1 m/s (normal < 2.8 m/s).
 
Right heart catheterization reveals a mean pulmonary arterial pressure of 26 mm Hg. What additional abnormal finding is likely to be seen in this patient?
A. Elevated pulmonary capillary wedge pressure
B. Mediastinal lymphadenopathy
C. Pulmonary arterial thrombosis
D. Reduced blood brain natriuretic peptide
E. Schistosomiasis infection
 
11. A 52-year-old woman presents with progressive dyspnea for 2 months. She does not have cough, hemoptysis, fever, chest pain or weight loss. She has a history of hypertension for 20 years. Her family history is positive for a few members with hypertension. She does not drink alcohol nor smoke cigarette. She has blood pressure of 152/95 mm Hg. Her other vital signs are within normal range. Physical examination reveals bilateral lower leg edema. She has a loud second heart sound, but no heart murmurs are heard. Pulmonary auscultation reveals rales in both lung bases. No hepatomegaly is noted. Her CBC, liver and renal function tests, and coagulation panels are within normal range. EKG reveals left ventricular hypertrophy. Echocardiogram reveals a peak tricuspid regurgitation velocity of 3.1 m/s (normal < 2.8 m/s). Right heart catheterization reveals a mean pulmonary arterial pressure of 26 mm Hg. What is the diagnosis?
A. Acute pulmonary embolism
B. Congenital tricuspid regurgitation
C. Cor pulmonale
D. Coronary heart disease
E. Pulmonary hypertension
 
12. A 52-year-old woman presents with progressive dyspnea for 2 months. She does not have cough, hemoptysis, fever, chest pain or weight loss. She has a history of hypertension for 20 years. Her family history is positive for a few members with hypertension. She does not drink alcohol nor smoke cigarette. She has blood pressure of 152/95 mm Hg. Her other vital signs are within normal range. Physical examination reveals bilateral lower leg edema. She has a loud second heart sound, but no heart murmurs are heard. Pulmonary auscultation reveals rales in both lung bases. No hepatomegaly is noted. Her CBC, liver and renal function tests, and coagulation panels are within normal range. EKG reveals left ventricular hypertrophy. Echocardiogram reveals a peak tricuspid regurgitation velocity of 3.1 m/s (normal < 2.8 m/s). Right heart catheterization reveals a mean pulmonary arterial pressure of 26 mm Hg. What is the most likely cause of her right heart catheterization findings?
A. Chronic obstructive pulmonary disease
B. Congenital tricuspid abnormality
C. Left heart dysfunction
D. Pulmonary artery malformation
E. Recurrent pulmonary embolism
 
13. A 52-year-old woman presents with progressive dyspnea for 2 months. She does not have cough, hemoptysis, fever, chest pain or weight loss. She has a history of hypertension for 20 years. Her family history is positive for a few members with hypertension. She does not drink alcohol nor smoke cigarette. She has blood pressure of 152/95 mm Hg. Her other vital signs are within normal range. Physical examination reveals bilateral lower leg edema. She has a loud second heart sound, but no heart murmurs are heard. Pulmonary auscultation reveals rales in both lung bases. No hepatomegaly is noted. Her CBC, liver and renal function tests, and coagulation panels are within normal range. EKG reveals left ventricular hypertrophy. Echocardiogram reveals a peak tricuspid regurgitation velocity of 3.1 m/s (normal < 2.8 m/s). Right heart catheterization reveals a mean pulmonary arterial pressure of 26 mm Hg. What is the most likely cause of her elevated pulmonary artery pressure?
A. Elevated left heart filling pressure
B. Pulmonary arteriole intima hyperplasia
C. Pulmonary embolism
D. Pulmonary valve stenosis
E. Right heart dysfunction
 
 
14. Use this case for next 6 questions. A 35-year-old woman presents with mild chest pain, progressive dyspnea and lower extremity edema for 2 months. She has a history of deep vein thrombosis, non-ischemic cardiomyopathy, chronic coughing, heart palpitation, Hashimoto thyroiditis, and is currently taking thyroxines and warfarin. She does not have history of hemoptysis, night sweating or weight loss. Her social and family histories are unremarkable. She appears mild anxious with an irregularly irregular heart rate at 120 beats per minute, and oxygen saturation of 80%. Her other vital signs are within normal ranges. Physical examination reveals jugular venous distention and bilateral lower extremity edema. Chest auscultation reveals loud P2. No murmur of heart is noted. Low-pitched murmur are noted bilaterally at the middle and lower lung fields. No crackles nor wheezing are noted. Her hemoglobin, PT, aPTT, liver and renal functions tests are within normal range. What diagnostic test should be performed next?
A. Chest X-ray
B. Coronary angiogram
C. EKG
D. Thyroid sonography
E. V/Q scan
 
15. A 35-year-old woman presents with mild chest pain, progressive dyspnea and lower extremity edema for 2 months. She has a history of deep vein thrombosis, non-ischemic cardiomyopathy, chronic coughing, heart palpitation, Hashimoto thyroiditis, and is currently taking thyroxines and warfarin. She does not have history of hemoptysis, night sweating or weight loss. Her social and family histories are unremarkable. She appears mild anxious with an irregularly irregular heart rate at 120 beats per minute, and oxygen saturation of 80%. Her other vital signs are within normal ranges. Physical examination reveals jugular venous distention and bilateral lower extremity edema. Chest auscultation reveals loud P2. No murmur of heart is noted. Low-pitched murmur are noted bilaterally at the middle and lower lung fields. No crackles nor wheezing are noted. Her hemoglobin, PT, aPTT, liver and renal functions tests are within normal range.  V/Q scan reveals many mismatch perfusion defects.
 
What exam is most likely to confirm the underlying disorder?
A. CT-guided lung biopsy
B. CT Pulmonary angiogram
C. Magnetic resonance pulmonary angiogram
D. Serum D-dimer
E. Ultrasound-guided thyroid biopsy
 
16. A 35-year-old woman presents with mild chest pain, progressive dyspnea and lower extremity edema for 2 months. She has a history of deep vein thrombosis, non-ischemic cardiomyopathy, chronic coughing, heart palpitation, Hashimoto thyroiditis, and is currently taking thyroxines and warfarin. She does not have history of hemoptysis, night sweating or weight loss. Her social and family histories are unremarkable. She appears mild anxious with an irregularly irregular heart rate at 120 beats per minute, and oxygen saturation of 80%. Her other vital signs are within normal ranges. Physical examination reveals jugular venous distention and bilateral lower extremity edema. Chest auscultation reveals loud P2. No murmur of heart is noted. Low-pitched murmur are noted bilaterally at the middle and lower lung fields. No crackles nor wheezing are noted. Her hemoglobin, PT, aPTT, liver and renal functions tests are within normal range. V/Q scan reveals many mismatch perfusion defects. Pulmonary angiogram reveals multiple filling defects bilaterally, involving right middle lobe and both lower lobes.
 
What exam is most likely to confirm the diagnosis?
A. Chest-MRI
B. CT-guided lung biopsy
C. Pulmonary angiogram
D. Right heart catheterization
E. Ultrasound-guided thyroid biopsy
 
17. A 35-year-old woman presents with mild chest pain, progressive dyspnea and lower extremity edema for 2 months. She has a history of deep vein thrombosis, non-ischemic cardiomyopathy, chronic coughing, heart palpitation, Hashimoto thyroiditis, and is currently taking thyroxines and warfarin. She does not have history of hemoptysis, night sweating or weight loss. Her social and family histories are unremarkable. She appears mild anxious with an irregularly irregular heart rate at 120 beats per minute, and oxygen saturation of 80%. Her other vital signs are within normal ranges. Physical examination reveals jugular venous distention and bilateral lower extremity edema. Chest auscultation reveals loud P2. No murmur of heart is noted. Low-pitched murmur are noted bilaterally at the middle and lower lung fields. No crackles nor wheezing are noted. Her hemoglobin, PT, aPTT, liver and renal functions tests are within normal range. V/Q scan reveals many mismatch perfusion defects. Pulmonary angiogram reveals multiple filling defects bilaterally, involving right middle lobe and both lower lobes. What additional test/exam result is likely to be elevated?
A. Absolute eosinophil count
B. Fibrinogen
C. D-dimer
D. Platelet count
E. Pulmonary capillary wedge pressure
F. Troponin
 
18. A 35-year-old woman presents with mild chest pain, progressive dyspnea and lower extremity edema for 2 months. She has a history of deep vein thrombosis, non-ischemic cardiomyopathy, chronic coughing, heart palpitation, Hashimoto thyroiditis, and is currently taking thyroxines and warfarin. She does not have history of hemoptysis, night sweating or weight loss. Her social and family histories are unremarkable. She appears mild anxious with an irregularly irregular heart rate at 120 beats per minute, and oxygen saturation of 80%. Her other vital signs are within normal ranges. Physical examination reveals jugular venous distention and bilateral lower extremity edema. Chest auscultation reveals loud P2. No murmur of heart is noted. Low-pitched murmur are noted bilaterally at the middle and lower lung fields. No crackles nor wheezing are noted. Her hemoglobin, PT, aPTT, liver and renal functions tests are within normal range. V/Q scan reveals many mismatch perfusion defects. Pulmonary angiogram reveals multiple filling defects bilaterally, involving right middle lobe and both lower lobes. Right heart catheterization reveals a mean pulmonary artery pressure of 27 mm Hg, and a pulmonary capillary wedge pressure of 12 mm Hg. What is the diagnosis?
A. Bronchopneumonia
B. Cor pulmonale
C. Left heart failure
D. Pulmonary hypertension
E. Superior vena cava syndrome
 
19. A 35-year-old woman presents with mild chest pain, progressive dyspnea and lower extremity edema for 2 months. She has a history of deep vein thrombosis, non-ischemic cardiomyopathy, chronic coughing, heart palpitation, Hashimoto thyroiditis, and is currently taking thyroxines and warfarin. She does not have history of hemoptysis, night sweating or weight loss. Her social and family histories are unremarkable. She appears mild anxious with an irregularly irregular heart rate at 120 beats per minute, and oxygen saturation of 80%. Her other vital signs are within normal ranges. Physical examination reveals jugular venous distention and bilateral lower extremity edema. Chest auscultation reveals loud P2. No murmur of heart is noted. Low-pitched murmur are noted bilaterally at the middle and lower lung fields. No crackles nor wheezing are noted. Her hemoglobin, PT, aPTT, liver and renal functions tests are within normal range. V/Q scan reveals many mismatch perfusion defects. Pulmonary angiogram reveals multiple filling defects bilaterally, involving right middle lobe and both lower lobes. Right heart catheterization reveals a mean pulmonary artery pressure of 27 mm Hg, and a pulmonary capillary wedge pressure of 12 mm Hg. What is the pathological basis for her presentations?
A. Congenital pulmonary artery stenosis
B. Left ventricular hypertrophy
C. Organized thromboemboli in pulmonary arteries
D. Pulmonary interstitial fibrosis
E. Singular millimetric fibrovascular lesions
 
 
20. Use this case for next 5 questions. A 31-year-old man presents with persistent shortness of breath for 3 months. He does not have other symptoms. His past medical history is unremarkable. He has a 10 pack-year history of cigarette smoking. He moved to the States 1 year ago from west Africa. His vital signs are within normal range. Physical examination reveals loud P2 and left parasternal heave. No other significant abnormalities are noted. His CBC, liver and renal function tests are within normal range. Chest X-ray reveals dilated pulmonary trunks, with reduced vascular shadow at periphery. What exam should be performed next?
A. Blood pH
B. Coronary angiogram
C. Echocardiogram
D. Liver function tests
E. Renal function tests
 
21. A 31-year-old man presents with persistent shortness of breath for 3 months. He does not have other symptoms. His past medical history is unremarkable. He has a 10 pack-year history of cigarette smoking. He moved to the States 1 year ago from west Africa. His vital signs are within normal range. Physical examination reveals loud P2 and left parasternal heave. No other significant abnormalities are noted. His CBC, liver and renal function tests are within normal range. Chest X-ray reveals dilated pulmonary trunks, with reduced vascular shadow at periphery.  
 
Echocardiogram reveals dilated right ventricle. No abnormality is noted in his left heart. What exam should be performed next?
A. Chest-MRI
B. Coagulation panel
C. CT-guided lung biopsy
D. Pulmonary angiogram
E. Right heart catheterization
 
22. A 31-year-old man presents with persistent shortness of breath for 3 months. He does not have other symptoms. His past medical history is unremarkable. He has a 10 pack-year history of cigarette smoking. He moved to the States 1 year ago from west Africa. His vital signs are within normal range. Physical examination reveals loud P2 and left parasternal heave. No other significant abnormalities are noted. His CBC, liver and renal function tests are within normal range. Chest X-ray reveals dilated pulmonary trunks, with reduced vascular shadow at periphery. Echocardiogram reveals dilated right ventricle. No abnormality is noted in his left heart. His PT, aPTT and D-dimer are within normal range.
Right heart catheterization reveals a mean pulmonary artery pressure of 28 mm Hg. What is most likely the diagnosis?
A. Chronic thromboembolic pulmonary hypertension
B. Primary pulmonary hypertension
C. Pulmonary hypertension due to left-sided heart disease
D. Pulmonary hypertension due to lung diseases and/or hypoxia
E. Pulmonary hypertension due to pulmonary artery stenosis
 
23. A 31-year-old man presents with persistent shortness of breath for 3 months. He does not have other symptoms. His past medical history is unremarkable. He has a 10 pack-year history of cigarette smoking. He moved to the States 1 year ago from west Africa. His vital signs are within normal range. Physical examination reveals loud P2 and left parasternal heave. No other significant abnormalities are noted. His CBC, liver and renal function tests are within normal range. Chest X-ray reveals dilated pulmonary trunks, with reduced vascular shadow at periphery. Echocardiogram reveals dilated right ventricle. No abnormality is noted in his left heart. His PT, aPTT and D-dimer are within normal range.
Right heart catheterization reveals a mean pulmonary artery pressure of 28 mm Hg. What exam should be performed next?
A. Arterial blood gas test
B. Chest-MRI
C. Fecal parasite studies
D. Pulmonary angiogram
E. Serum troponin
 
24. A 31-year-old man presents with persistent shortness of breath for 3 months. He does not have other symptoms. His past medical history is unremarkable. He has a 10 pack-year history of cigarette smoking. He moved to the States 1 year ago from west Africa. His vital signs are within normal range. Physical examination reveals loud P2 and left parasternal heave. No other significant abnormalities are noted. His CBC, liver and renal function tests are within normal range. Chest X-ray reveals dilated pulmonary trunks, with reduced vascular shadow at periphery. Echocardiogram reveals dilated right ventricle. No abnormality is noted in his left heart. His PT, aPTT and D-dimer are within normal range. Right heart catheterization reveals a mean pulmonary artery pressure of 28 mm Hg. What pathological changes are most likely associated with his presentations?
A. Alveolar neutrophilic infiltration
B. Left ventricular hypertrophy
C. Media proliferation involving small pulmonary arteries
D. Organized thrombi in pulmonary arterioles
E. Pulmonary interstitial fibrosis
 
 
25. Use this case for next 3 questions. A 42-year-old woman presents with shortness of breath after exertion for 3 months. She has intermittent cough in the last 8 years. She does not have fever, chest pain nor palpitation. She had a history of pulmonary embolism 2 year ago. Her past medical history is otherwise unremarkable. Her vital signs are within normal range. Physical exam reveals bilateral jugular vein distention and 3+ edema involving both ankles. A palpable heave is noted at the fifth intercostal space just left to the sternum. There is a holosystolic murmur loudest to the left lower sternal border. She has accentuated P2.  The liver is 5 cm below the costal margin. No evidence of ascites is noted. Her CBC is within normal range. She has a D-dimer of 2.5 mcg/ml (normal ≤ 0.50 mcg/ml). What exam is likely to confirm the diagnosis of her lung abnormalities?
A. Chest-MRI
B. CT-guided lung biopsy
C. Echocardiogram
D. Pulmonary angiogram
E. Right heart catheterization
 
26. A 42-year-old woman presents with shortness of breath after exertion for 3 months. She has intermittent cough in the last 8 years. She does not have fever, chest pain nor palpitation. She had a history of pulmonary embolism 2 year ago. Her past medical history is otherwise unremarkable. Her vital signs are within normal range. Physical exam reveals bilateral jugular vein distention and 3+ edema involving both ankles. A palpable heave is noted at the fifth intercostal space just left to the sternum. There is a holosystolic murmur loudest to the left lower sternal border. She has accentuated P2.  The liver is 5 cm below the costal margin. No evidence of ascites is noted. Her CBC is within normal range. She has a D-dimer of 2.5 mcg/ml (normal ≤ 0.50 mcg/ml).
 
Her echocardiogram reveals dilated right ventricle. Pulmonary angiogram reveal multiple filling defects bilaterally. What is the diagnosis?
A. Cardiomyopathy
B. Congenital tricuspid regurgitation
C. Cor pulmonale
D. Primary pulmonary hypertension
E. Pulmonary valve stenosis
 
27. A 42-year-old woman presents with shortness of breath after exertion for 3 months. She has intermittent cough in the last 8 years. She does not have fever, chest pain nor palpitation. She had a history of pulmonary embolism 2 year ago. Her past medical history is otherwise unremarkable. Her vital signs are within normal range. Physical exam reveals bilateral jugular vein distention and 3+ edema involving both ankles. A palpable heave is noted at the fifth intercostal space just left to the sternum. There is a holosystolic murmur loudest to the left lower sternal border. She has accentuated P2.  The liver is 5 cm below the costal margin. No evidence of ascites is noted. Her CBC is within normal range. She has a D-dimer of 2.5 mcg/ml (normal ≤ 0.50 mcg/ml). Her echocardiogram reveals dilated right ventricle. Pulmonary angiogram reveal multiple filling defects bilaterally. What is the underlying cause of her presentation?
A. Bacterial infection
B. Chronic lung diseases
C. Left ventricular dysfunction
D. Pulmonary valve stenosis
E. Recurrent pulmonary thromboembolism
 
 
28. Use this case for next 2 questions. A 35-year-old woman presents with progressively worsening shortness of breath on exertion for 3 months. She does not have fevers, chills, or cough. She does not smoke cigarettes nor drink alcohol. She has two paternal side aunts with similar presentations. Physical examination reveals loud S2. No other abnormalities are noted. Her CBC, coagulation panels, liver and renal function tests are within normal ranges. Chest x-ray reveals dilated pulmonary trunk and reduced periphery vascular shadows. Her EKG reveals no significant abnormalities. Echocardiogram reveals a slightly dilated right ventricle and an estimated pulmonary artery pressure of 31 mm Hg. What is the most likely diagnosis?
A. Chronic thromboembolic pulmonary hypertension
B. Cor pulmonale
C. Primary pulmonary hypertension
D. Pulmonary hypertension due to left-sided heart disease
E. Pulmonary hypertension due to lung diseases and/or hypoxia
 
29. A 35-year-old woman presents with progressively worsening shortness of breath on exertion for 3 months. She does not have fevers, chills, or cough. She does not smoke cigarettes nor drink alcohol. She has two paternal side aunts with similar presentations. Physical examination reveals loud S2. No other abnormalities are noted. Her CBC, coagulation panels, liver and renal function tests are within normal ranges. Chest x-ray reveals dilated pulmonary trunk and reduced periphery vascular shadows. Her EKG reveals no significant abnormalities. Echocardiogram reveals a slightly dilated right ventricle and an estimated pulmonary artery pressure of 31 mm Hg. What gene is most likely abnormal in this patient?
A. BMPR2
B. Factor V
C. PRKAR1A
D. TSC1
E. VHL
 

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