Pulmonary Hypertension
Kiely DG, Elliot CA, Sabroe I, Condliffe R. Pulmonary hypertension: diagnosis and management. BMJ. 2013;346:f2028
Question 1: Epidemiology (1)
A 69 year-old male with progressive breathlessness is admitted under the medical assessment and planning unit (MAPU) with progressive breathlessness for investigation. He had an unprovoked pulmonary embolus 3 years ago that was treated with 6 months of warfarin. Since that time, he has noted his breathlessness to worsen progressively. Further complaints include intermittent dizziness and syncope. He has noted an increase in his abdominal girth with accompanying leg swelling. He is now largely bed-bound and is completely dependent on his family for his activities of daily living. He has a loud P2, an ejection systolic murmur over the tricuspid region and a right ventricular heave. His JVP is elevated without ‘a’ waves but prominent ‘v’ waves. He has shifting dullness and peripheral oedema up to mid thigh. His ECG shows signs of right ventricular hypertrophy, and his ECHO estimates a systolic pulmonary pressure of 55mmHg using the estimated tricuspid jet velocity in ‘M’ mode.
With regards to his most likely diagnosis, what is the most CORRECT statement?
(A) Idiopathic pulmonary arterial hypertension (IPAH) patients most likely carry the mutation for Bone Morphogenetic Receptor 2
(B) The expected survival of a patient who responds to inhaled nitric oxide with a drop in their mean pulmonary arterial pressure of 8mmHg is highly favourable if they are placed on high dose nifedipine or diltiazem
(C) 10% of patients will get pulmonary hypertension complicating a pulmonary embolus
(D) The treatment of patients with pulmonary artery hypertension secondary to obesity hypoventilation syndrome is similar to that of pulmonary artery hypertension secondary to chronic thromboembolic disease
(E) The mean age of diagnosis if idiopathic pulmonary arterial hypertension (IPAH) is 55 years with a median survival that is improving, however 5 year survival being only 2.8 years
Question 2: Epidemiology and prognostic factors
Which of these following factors is NOT a known adverse prognostic feature of pulmonary arterial hypertension
(A) Echocardiographic findings of large right atrial diameter, diastolic septal shifting and pericardial effusions
(B) Hypocapnia
(C) ECG findings of atrial fibrillation, atrial flutter or prolonged QRS duration
(D) Female gender
(E) Six minute walk test
Question 3: Clinical Diagnosis
On the Jugular venous pressure trace, what feature is associated with pulmonary arterial hypertension?
(A) Absent ‘a’ waves
(B) Prolonged ‘x’ and ‘y’ descent
(C) Kausmalls sign
(D) Cannon ‘a’ waves
(E) Raised ‘v’ waves
Question 4: Clinical Diagnosis
Which of the following clinical signs are NOT associated with pulmonary arterial hypertension?
(A) Right ventricular lift
(B) Pulsatile liver, ascites and peripheral oedema
(C) Early decrescendo diastolic murmur with pre-systolic blowing murmur
(D) Right ventricular third heart sound
(E) Paradoxically split second heart sound with palpable S2
Question 5: Clinical Diagnosis
What is the most common arrhythmia associated with pulmonary arterial hypertension?
(A) Atrial Fibrillation
(B) Atrial Flutter
(C) Atrio-Ventricular Nodal Re-entrant Tachycardia
(D) Idioventricular Rhythm
(E) Sinus Bradycardia
Question 6: Clinical Diagnosis
A 69 year-old male with progressive breathlessness is admitted under the medical assessment and planning unit (MAPU) with progressive breathlessness for investigation. He had an unprovoked pulmonary embolus 3 years ago that was treated with 6 months of warfarin. Since that time, he has noted his breathlessness to worsen progressively. Further complaints include intermittent dizziness and syncope. He has noted an increase in his abdominal girth with accompanying leg swelling. He is now largely bed-bound and is completely dependent on his family for his activities of daily living. He has a loud P2, an ejection systolic murmur over the tricuspid region and a right ventricular heave. His JVP is elevated without ‘a’ waves but prominent ‘v’ waves. He has shifting dullness and peripheral oedema up to mid thigh. His ECG shows signs of right ventricular hypertrophy, and his ECHO estimates a systolic pulmonary pressure of 55mmHg using the estimated tricuspid jet velocity in ‘M’ mode.
What chest X-ray imaging sign would be LEAST associated with pulmonary hypertension
(A) Cardiomegaly
(B) Pulmonary Arterial Pruning
(C) Right Upper Lobe consolidation with air-bronchograms
(D) Bi-basal predominant interstitial infiltrates resembling fibrosis
(E) Hampton’s hump
Question 7: Clinical Diagnosis
A 28 year-old man is suspected of having idiopathic pulmonary artery hypertension with a JVP measuring 5cm above sternal notch. He has an echocardiogram performed. The views were of sufficient quality, enabling the sonographer to calculate the following parameters:
Tricuspid regurgitant jet velocity: 4m.s-1
Left ventricular Ejection Fraction using Simpsons Method: 58%
Fractional shortening: 37%
What is the BEST fit for calculated parameters of Pressure gradient (dP), Right atrial Pressure, Systolic Pressure and Diagnosis
Answer
(A) Pressure gradient (dP) 64, RAP 10, sPAP 54, Diagnosis: Pulmonary hypertension
(B) Pressure gradient (dP)64, RAP 10, sPAP 74, Diagnosis: Undetermined
(C) Pressure gradient (dP) 64, RAP 5, sPAP 69, Diagnosis: Undetermined
(D) Pressure gradient (dP) 48, RAP 5, sPAP 53, Diagnosis: Pulmonary Hypertension
(E) Pressure gradient (dP) 48, RAP 10, sPAP 58, Diagnosis: Pulmonary Hypertension
Question 9: Categories of pulmonary hypertension
Once the diagnosis of pulmonary arterial hypertension is distinguished from pulmonary venous hypertension, which of the below options does NOT adequately help distinguish a cause
(A) ANA and interstitial lung disease
(B) V/Q and CTPEH
(C) anti-scl70 and diffuse scleroderma
(D) anti-centromere and limited scleroderma
(E) HIV third generation antigen/ antibody and HIV
Question 10: Causes
Which of the following medications are NOT associated with idiopathic pulmonary arterial hypertension
(A) dasatinib
(B) appetite suppressants
(C) St Johns wort
(D) chronic cocaine use
(E) obinutuzumab
Question 11: Treatment
A 68 year-old male with idiopathic pulmonary hypertension is undergoing consideration for treatment. Which of the following statements is FALSE about the role of anti-coagulation.
(A) Warfarin is the anticoagulation of choice because other studies are lacking
(B) A meta-analysis of seven observational studies has shown a benefit favouring anti-coagulation in terms of mortality
(C) There is no difference in bleeding risk in patients with PAH secondary to connective tissue disease in comparison to patients with idiopathic pulmonary hypertension
(D) There is no evidence for use of anticoagulation in PAH due to anorexigens
(E) The rationale for anti-coagulation is due to sluggish pulmonary blood flow leading to a predisposition for thrombosis on the background of poor physiological reserve
Question 12: Treatment
Which patient group is most likely to respond to the nitrous oxide vasoreactivity test?
(A) Pulmonary arterial hypertension secondary to non-specific interstitial pneumonia
(B) Pulmonary arterial hypertension secondary to drugs such as anorexigen
(C) Pulmonary arterial hypertension secondary to HIV
(D) Pulmonary arterial hypertension secondary to schistosomiasis
(E) Pulmonary arterial hypertension secondary to PTPN22a mutation
Question 13: Treatment
What is the mortality of a patient who has pulmonary arterial hypertension who falls pregnant?
(A) 17 – 33%
(B) 2 – 12%
(C) 35 – 60%
(D) 50 – 70%
(E) 60 – 85%
Question 14: Treatment
Which of the following statements is INCORRECT
(A) Bosentan and ambrisentan are endothelin receptor antagonists, functionally causing a pulmonary vasodilated state, with Bosentan causing reversible liver function abnormalities in 10% requiring monthly LFT monitoring
(B) Sildanefil and tadalafil inhibit phosphodiesterase 5, which causes increased cyclic GMP via nitrous oxide therefore causing pulmonary smooth muscle vasodilation. Its use is contra-indicated with other nitrates
(C) One criterion of the PBS states that right heart catheterisation should be used and the criterion is that the mean pulmonary arterial pressure is greater than 25 mmHg with the pulmonary capillary wedge pressure less than 15mmHg
(D) Prostanoids are prostacyclins that cause pulmonary arterial vasodilation with added anti-proliferative effects
(E) Riociguat cannot be used for the treatment of pulmonary artery hypertension secondary to chronic thromboembolic disease
A 69 year-old male with progressive breathlessness is admitted under the medical assessment and planning unit (MAPU) with progressive breathlessness for investigation. He had an unprovoked pulmonary embolus 3 years ago that was treated with 6 months of warfarin. Since that time, he has noted his breathlessness to worsen progressively. Further complaints include intermittent dizziness and syncope. He has noted an increase in his abdominal girth with accompanying leg swelling. He is now largely bed-bound and is completely dependent on his family for his activities of daily living. He has a loud P2, an ejection systolic murmur over the tricuspid region and a right ventricular heave. His JVP is elevated without ‘a’ waves but prominent ‘v’ waves. He has shifting dullness and peripheral oedema up to mid thigh. His ECG shows signs of right ventricular hypertrophy, and his ECHO estimates a systolic pulmonary pressure of 55mmHg using the estimated tricuspid jet velocity in ‘M’ mode.
With regards to his most likely diagnosis, what is the most CORRECT statement?
(A) Idiopathic pulmonary arterial hypertension (IPAH) patients most likely carry the mutation for Bone Morphogenetic Receptor 2
(B) The expected survival of a patient who responds to inhaled nitric oxide with a drop in their mean pulmonary arterial pressure of 8mmHg is highly favourable if they are placed on high dose nifedipine or diltiazem
(C) 10% of patients will get pulmonary hypertension complicating a pulmonary embolus
(D) The treatment of patients with pulmonary artery hypertension secondary to obesity hypoventilation syndrome is similar to that of pulmonary artery hypertension secondary to chronic thromboembolic disease
(E) The mean age of diagnosis if idiopathic pulmonary arterial hypertension (IPAH) is 55 years with a median survival that is improving, however 5 year survival being only 2.8 years
Question 2: Epidemiology and prognostic factors
Which of these following factors is NOT a known adverse prognostic feature of pulmonary arterial hypertension
(A) Echocardiographic findings of large right atrial diameter, diastolic septal shifting and pericardial effusions
(B) Hypocapnia
(C) ECG findings of atrial fibrillation, atrial flutter or prolonged QRS duration
(D) Female gender
(E) Six minute walk test
Question 3: Clinical Diagnosis
On the Jugular venous pressure trace, what feature is associated with pulmonary arterial hypertension?
(A) Absent ‘a’ waves
(B) Prolonged ‘x’ and ‘y’ descent
(C) Kausmalls sign
(D) Cannon ‘a’ waves
(E) Raised ‘v’ waves
Question 4: Clinical Diagnosis
Which of the following clinical signs are NOT associated with pulmonary arterial hypertension?
(A) Right ventricular lift
(B) Pulsatile liver, ascites and peripheral oedema
(C) Early decrescendo diastolic murmur with pre-systolic blowing murmur
(D) Right ventricular third heart sound
(E) Paradoxically split second heart sound with palpable S2
Question 5: Clinical Diagnosis
What is the most common arrhythmia associated with pulmonary arterial hypertension?
(A) Atrial Fibrillation
(B) Atrial Flutter
(C) Atrio-Ventricular Nodal Re-entrant Tachycardia
(D) Idioventricular Rhythm
(E) Sinus Bradycardia
Question 6: Clinical Diagnosis
A 69 year-old male with progressive breathlessness is admitted under the medical assessment and planning unit (MAPU) with progressive breathlessness for investigation. He had an unprovoked pulmonary embolus 3 years ago that was treated with 6 months of warfarin. Since that time, he has noted his breathlessness to worsen progressively. Further complaints include intermittent dizziness and syncope. He has noted an increase in his abdominal girth with accompanying leg swelling. He is now largely bed-bound and is completely dependent on his family for his activities of daily living. He has a loud P2, an ejection systolic murmur over the tricuspid region and a right ventricular heave. His JVP is elevated without ‘a’ waves but prominent ‘v’ waves. He has shifting dullness and peripheral oedema up to mid thigh. His ECG shows signs of right ventricular hypertrophy, and his ECHO estimates a systolic pulmonary pressure of 55mmHg using the estimated tricuspid jet velocity in ‘M’ mode.
What chest X-ray imaging sign would be LEAST associated with pulmonary hypertension
(A) Cardiomegaly
(B) Pulmonary Arterial Pruning
(C) Right Upper Lobe consolidation with air-bronchograms
(D) Bi-basal predominant interstitial infiltrates resembling fibrosis
(E) Hampton’s hump
Question 7: Clinical Diagnosis
A 28 year-old man is suspected of having idiopathic pulmonary artery hypertension with a JVP measuring 5cm above sternal notch. He has an echocardiogram performed. The views were of sufficient quality, enabling the sonographer to calculate the following parameters:
Tricuspid regurgitant jet velocity: 4m.s-1
Left ventricular Ejection Fraction using Simpsons Method: 58%
Fractional shortening: 37%
What is the BEST fit for calculated parameters of Pressure gradient (dP), Right atrial Pressure, Systolic Pressure and Diagnosis
Answer
(A) Pressure gradient (dP) 64, RAP 10, sPAP 54, Diagnosis: Pulmonary hypertension
(B) Pressure gradient (dP)64, RAP 10, sPAP 74, Diagnosis: Undetermined
(C) Pressure gradient (dP) 64, RAP 5, sPAP 69, Diagnosis: Undetermined
(D) Pressure gradient (dP) 48, RAP 5, sPAP 53, Diagnosis: Pulmonary Hypertension
(E) Pressure gradient (dP) 48, RAP 10, sPAP 58, Diagnosis: Pulmonary Hypertension
Question 9: Categories of pulmonary hypertension
Once the diagnosis of pulmonary arterial hypertension is distinguished from pulmonary venous hypertension, which of the below options does NOT adequately help distinguish a cause
(A) ANA and interstitial lung disease
(B) V/Q and CTPEH
(C) anti-scl70 and diffuse scleroderma
(D) anti-centromere and limited scleroderma
(E) HIV third generation antigen/ antibody and HIV
Question 10: Causes
Which of the following medications are NOT associated with idiopathic pulmonary arterial hypertension
(A) dasatinib
(B) appetite suppressants
(C) St Johns wort
(D) chronic cocaine use
(E) obinutuzumab
Question 11: Treatment
A 68 year-old male with idiopathic pulmonary hypertension is undergoing consideration for treatment. Which of the following statements is FALSE about the role of anti-coagulation.
(A) Warfarin is the anticoagulation of choice because other studies are lacking
(B) A meta-analysis of seven observational studies has shown a benefit favouring anti-coagulation in terms of mortality
(C) There is no difference in bleeding risk in patients with PAH secondary to connective tissue disease in comparison to patients with idiopathic pulmonary hypertension
(D) There is no evidence for use of anticoagulation in PAH due to anorexigens
(E) The rationale for anti-coagulation is due to sluggish pulmonary blood flow leading to a predisposition for thrombosis on the background of poor physiological reserve
Question 12: Treatment
Which patient group is most likely to respond to the nitrous oxide vasoreactivity test?
(A) Pulmonary arterial hypertension secondary to non-specific interstitial pneumonia
(B) Pulmonary arterial hypertension secondary to drugs such as anorexigen
(C) Pulmonary arterial hypertension secondary to HIV
(D) Pulmonary arterial hypertension secondary to schistosomiasis
(E) Pulmonary arterial hypertension secondary to PTPN22a mutation
Question 13: Treatment
What is the mortality of a patient who has pulmonary arterial hypertension who falls pregnant?
(A) 17 – 33%
(B) 2 – 12%
(C) 35 – 60%
(D) 50 – 70%
(E) 60 – 85%
Question 14: Treatment
Which of the following statements is INCORRECT
(A) Bosentan and ambrisentan are endothelin receptor antagonists, functionally causing a pulmonary vasodilated state, with Bosentan causing reversible liver function abnormalities in 10% requiring monthly LFT monitoring
(B) Sildanefil and tadalafil inhibit phosphodiesterase 5, which causes increased cyclic GMP via nitrous oxide therefore causing pulmonary smooth muscle vasodilation. Its use is contra-indicated with other nitrates
(C) One criterion of the PBS states that right heart catheterisation should be used and the criterion is that the mean pulmonary arterial pressure is greater than 25 mmHg with the pulmonary capillary wedge pressure less than 15mmHg
(D) Prostanoids are prostacyclins that cause pulmonary arterial vasodilation with added anti-proliferative effects
(E) Riociguat cannot be used for the treatment of pulmonary artery hypertension secondary to chronic thromboembolic disease
References:
1. Kiely DG, Elliot CA, Sabroe I, Condliffe R. Pulmonary hypertension: diagnosis and management. BMJ. 2013;346:f2028.
2. Hoeper MM, Pletz MW, Golpon H, Welte T. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2007;29(5):944-50.
3. Raymond RJ, Hinderliter AL, Willis PW, Ralph D, Caldwell EJ, Williams W, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002;39(7):1214-9.
4. Johnson SR, Mehta S, Granton JT. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J. 2006;28(5):999-1004.
5. Task Force for D, Treatment of Pulmonary Hypertension of European Society of C, European Respiratory S, International Society of H, Lung T, Galie N, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34(6):1219-63.
6. Simonneau G, D'Armini AM, Ghofrani HA, Grimminger F, Hoeper MM, Jansa P, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45(5):1293-302.
7. Galie N, Barbera JA, Frost AE, Ghofrani HA, Hoeper MM, McLaughlin VV, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med. 2015;373(9):834-44.
1. Kiely DG, Elliot CA, Sabroe I, Condliffe R. Pulmonary hypertension: diagnosis and management. BMJ. 2013;346:f2028.
2. Hoeper MM, Pletz MW, Golpon H, Welte T. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2007;29(5):944-50.
3. Raymond RJ, Hinderliter AL, Willis PW, Ralph D, Caldwell EJ, Williams W, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002;39(7):1214-9.
4. Johnson SR, Mehta S, Granton JT. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J. 2006;28(5):999-1004.
5. Task Force for D, Treatment of Pulmonary Hypertension of European Society of C, European Respiratory S, International Society of H, Lung T, Galie N, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34(6):1219-63.
6. Simonneau G, D'Armini AM, Ghofrani HA, Grimminger F, Hoeper MM, Jansa P, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45(5):1293-302.
7. Galie N, Barbera JA, Frost AE, Ghofrani HA, Hoeper MM, McLaughlin VV, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med. 2015;373(9):834-44.
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