Persistent Pulmonary Hypertension — MCQs
Drug not used in pulmonary hypertension is:
A 40F with progressive exertional dyspnea and cyanosis. Physical examination reveals digital clubbing and a loud P2 heart sound. Most appropriate next step in diagnosis?
Which of the following is not typically seen on a chest X-ray in pulmonary artery hypertension?
Child with PDA will NOT have:
A 30-year-old woman presents with shortness of breath, cyanosis, and a loud S2 sound on auscultation. Which of the following is the most likely diagnosis?
Which of the following is the best inotrope agent for use in right heart failure secondary to pulmonary hypertension?
With reference to Respiratory Distress Syndrome (RDS), which of the following statements is false?
Differential cyanosis occurs in which of the following diseases:
A newborn has been brought with seizures refractory to treatment and a continuous bruit through the anterior fontanelle. CT shows midline lesion with hypoechogenicity and dilated lateral ventricles. Most probable diagnosis is?
A dense persistent nephrogram may be seen in all of the following except:
Persistent Pulmonary Hypertension Indian Medical PG Practice Questions and MCQs
Question 1: Drug not used in pulmonary hypertension is:
- A. Endothelin receptor antagonist
- B. Prostacyclin
- C. Alpha blocker (Correct Answer)
- D. Calcium channel blocker
Explanation: ***Alpha blocker*** - Alpha-blockers primarily cause **systemic vasodilation** [1] and are not indicated for the specific pulmonary vascular remodeling and vasoconstriction seen in pulmonary hypertension. [2] - Their use could lead to an undesirable drop in **systemic blood pressure** [3] without adequately addressing the pulmonary arterial pressure. *Calcium channel blocker* - **Calcium channel blockers** (namely **dihydropyridines** like nifedipine and amlodipine) are used in a small subset of pulmonary hypertension patients who are **vasoreactive** on acute testing. - They work by relaxing pulmonary arterial smooth muscle, reducing **pulmonary vascular resistance**. *Endothelin receptor antagonist* - **Endothelin receptor antagonists** (e.g., bosentan, ambrisentan) block the effects of **endothelin-1**, a potent vasoconstrictor and smooth muscle proliferator involved in pulmonary hypertension. - They improve hemodynamics, exercise capacity, and clinical outcomes by preventing **vasoconstriction** and **vascular remodeling**. *Prostacyclin* - **Prostacyclin analogs** (e.g., epoprostenol, treprostinil) are potent **vasodilators** and inhibitors of platelet aggregation. - They are highly effective in treating severe pulmonary hypertension by relaxing pulmonary arteries and preventing **thrombosis**.
Question 2: A 40F with progressive exertional dyspnea and cyanosis. Physical examination reveals digital clubbing and a loud P2 heart sound. Most appropriate next step in diagnosis?
- A. Echocardiography (Correct Answer)
- B. Pulmonary function test
- C. Chest X-ray
- D. Electrocardiogram
Explanation: ***Echocardiography*** - The presence of **exertional dyspnea**, **cyanosis**, **digital clubbing**, and a **loud P2 heart sound** strongly suggests pulmonary hypertension [1]. - An **echocardiogram** is crucial for directly visualizing the heart chambers and great vessels, allowing for the estimation of **pulmonary artery pressures** and assessing right ventricular function, which is key in diagnosing and evaluating **pulmonary hypertension** [1]. *Pulmonary function test* - While pulmonary function tests (PFTs) assess lung mechanics and volumes, they primarily help diagnose **obstructive** or **restrictive lung diseases**. - PFTs do not directly measure **pulmonary artery pressures** or assess cardiac structure, which are central to the patient's presentation. *Chest X-ray* - A chest X-ray can show signs of **pulmonary hypertension** such as **enlarged pulmonary arteries** or **cardiomegaly** [1], [2]. - However, it provides limited information regarding cardiac function and **pulmonary artery pressures** and is less specific than an echocardiogram for initial diagnosis [1]. *Electrocardiogram* - An ECG can detect signs of **right ventricular hypertrophy** or **right axis deviation**, which may be present in **pulmonary hypertension** [2]. - However, it offers no direct information on **pulmonary artery pressures** or structural abnormalities of the heart chambers.
Question 3: Which of the following is not typically seen on a chest X-ray in pulmonary artery hypertension?
- A. Enlargement of central arteries
- B. Peripheral pruning
- C. Narrowing of central arteries (Correct Answer)
- D. None of the options
Explanation: ***Narrowing of central arteries*** - **Pulmonary artery hypertension** is characterized by the **enlargement of the central pulmonary arteries** due to increased pressure. - **Narrowing of central arteries** would contradict the hemodynamic changes seen in pulmonary hypertension. - This is the finding that is **NOT typically seen**, making this the correct answer. *Enlargement of central arteries* - This is a **hallmark radiographic finding** in pulmonary hypertension, reflecting the **dilatation of the main and proximal pulmonary arteries** due to increased pressure. - The **pulmonary artery segment becomes prominent**, often appearing convex on the left heart border. *Peripheral pruning* - This refers to the **abrupt tapering and loss of peripheral pulmonary vascular markings**, indicating reduced blood flow to the distal lung parenchyma. - It is a **common finding in advanced pulmonary hypertension**, as the distal vessels constrict and become obliterated. *None of the options* - This is incorrect since **narrowing of central arteries** is clearly not a typical finding in pulmonary hypertension.
Question 4: Child with PDA will NOT have:
- A. Necrotizing enterocolitis
- B. CO₂ washout
- C. Bounding pulses
- D. Pulmonary hemorrhage
Explanation: This question asks which finding is NOT associated with Patent Ductus Arteriosus (PDA). *Bounding pulses* - **Bounding pulses ARE characteristically present in PDA**, not absent - Result from wide pulse pressure due to diastolic run-off from aorta to pulmonary artery - This is a classic clinical sign of hemodynamically significant PDA ***Necrotizing enterocolitis - needs verification*** - PDA CAN be associated with NEC in premature infants - "Steal phenomenon" diverts blood from splanchnic circulation - However, NEC is multifactorial and not a direct consequence of PDA ***CO₂ washout - needs verification*** - Increased pulmonary blood flow from PDA can affect ventilation - May contribute to respiratory complications - The relationship is complex and context-dependent ***Pulmonary hemorrhage - needs verification*** - Large PDA with significant left-to-right shunt increases pulmonary blood flow - Can lead to pulmonary hemorrhage, especially in preterm infants - Increased pulmonary vascular pressure and volume cause capillary damage **Note:** This question has a structural issue - all listed options except bounding pulses (which IS present in PDA) CAN occur with PDA. The question requires review for medical accuracy and clarity.
Question 5: A 30-year-old woman presents with shortness of breath, cyanosis, and a loud S2 sound on auscultation. Which of the following is the most likely diagnosis?
- A. Aortic regurgitation
- B. Mitral stenosis
- C. Pulmonary hypertension (Correct Answer)
- D. Pericarditis
Explanation: ***Pulmonary hypertension*** - **Shortness of breath** and **cyanosis** indicate severe **hypoxia** and **reduced cardiac output**, which are hallmarks of high **pulmonary artery pressure**. [1] - A **loud S2** results from increased pressure in the pulmonary artery causing a forceful closure of the **pulmonic valve**. *Aortic regurgitation* - Characterized by a **diastolic murmur** and **bounding peripheral pulses**; **cyanosis** and a loud S2 are not typical features. - Patients often present with **dyspnea on exertion**, but not usually with prominent **cyanosis** unless heart failure is very advanced. *Mitral stenosis* - Typically presents with **dyspnea**, **cough**, and a **loud S1**, along with a **diastolic rumble** at the apex. - While it can lead to **pulmonary hypertension**, a loud S2 alone in the absence of a characteristic murmur does not make it the primary diagnosis. *Pericarditis* - Presents with sharp, pleuritic **chest pain** that improves with leaning forward, and often includes a **pericardial friction rub**. - **Shortness of breath** may occur due to pain or tamponade, but **cyanosis** and a loud S2 are not characteristic features of uncomplicated pericarditis.
Question 6: Which of the following is the best inotrope agent for use in right heart failure secondary to pulmonary hypertension?
- A. Milrinone (Correct Answer)
- B. Dobutamine
- C. Digoxin
- D. Dopamine
Explanation: ***Milrinone*** - Milrinone is a **phosphodiesterase-3 inhibitor** that increases myocardial contractility and causes **pulmonary and systemic vasodilation**. - Its vasodilatory effect is particularly beneficial in **pulmonary hypertension** as it can help reduce **pulmonary vascular resistance (PVR)**, a critical factor in right heart failure. - The combination of **positive inotropy** and **selective pulmonary vasodilation** makes it the optimal choice for right ventricular failure secondary to pulmonary hypertension. *Dobutamine* - Dobutamine is a **beta-1 agonist** that primarily increases myocardial contractility with some beta-2 mediated vasodilation. - While it improves cardiac output, its lesser effect on **pulmonary vascular resistance** compared to milrinone makes it less ideal for right heart failure specifically complicated by pulmonary hypertension. *Digoxin* - Digoxin is a **cardiac glycoside** that increases contractility but has a slow onset of action and a narrow therapeutic window, making it less suitable for acute management. - It does not significantly reduce **pulmonary vascular resistance** and is primarily used for chronic heart failure or rate control in atrial fibrillation. *Dopamine* - Dopamine is a **catecholamine** with dose-dependent effects: at moderate doses (5-10 mcg/kg/min), it acts as a **beta-1 agonist** providing inotropic support. - However, at higher doses it causes **alpha-adrenergic vasoconstriction** which can **increase pulmonary vascular resistance**, potentially worsening right heart failure in pulmonary hypertension. - Unlike milrinone, it lacks specific pulmonary vasodilatory properties beneficial for reducing RV afterload.
Question 7: With reference to Respiratory Distress Syndrome (RDS), which of the following statements is false?
- A. Leads to respiratory distress in premature infants
- B. Is less common in babies born to diabetic mothers (Correct Answer)
- C. Is treated by administering surfactant therapy
- D. Usually occurs in infants born before 34 weeks of gestation
Explanation: ***Is less common in babies born to diabetic mothers*** - Babies born to **diabetic mothers** are at an **increased risk** of Respiratory Distress Syndrome (RDS) due to delayed lung maturation caused by **hyperinsulinemia.** - Insulin inhibits the production of **surfactant**, a substance critical for reducing surface tension in the alveoli and preventing lung collapse. - This statement is **FALSE** - RDS is actually **MORE common** in infants of diabetic mothers. *Leads to respiratory distress in premature infants* - RDS is primarily a disease of **prematurity**, resulting from a deficiency of **surfactant** in the immature lungs. - This deficiency leads to widespread **atelectasis** (lung collapse), which causes breathing difficulties immediately or shortly after birth. - This statement is **TRUE**. *Is treated by administering surfactant therapy* - **Surfactant therapy** is a cornerstone of RDS treatment, often delivered via an **endotracheal tube**. - It works by replacing the deficient natural surfactant, thereby improving **lung compliance** and reducing the work of breathing. - This statement is **TRUE**. *Usually occurs in infants born before 34 weeks of gestation* - RDS predominantly affects infants born **before 34 weeks of gestation**, as their lungs are typically not mature enough to produce sufficient surfactant. - The risk **decreases significantly** with increasing gestational age, with full-term infants rarely developing the condition. - This statement is **TRUE**.
Question 8: Differential cyanosis occurs in which of the following diseases:
- A. PPHN (Correct Answer)
- B. Severe coarctation
- C. Interrupted aortic arch
- D. All of the options
Explanation: ***PPHN*** - **Persistent pulmonary hypertension of the newborn (PPHN)** is the **classic cause of differential cyanosis** in neonates. - High pulmonary vascular resistance leads to **suprasystemic pulmonary artery pressures**, causing **right-to-left shunting** across the **patent ductus arteriosus (PDA)**. - Desaturated blood from the pulmonary artery shunts into the **descending aorta** (post-ductal), perfusing the lower body and lower extremities with poorly oxygenated blood. - The upper extremities receive well-oxygenated blood from the **ascending aorta** (pre-ductal), resulting in **higher oxygen saturation in the right hand compared to the feet** (pre-ductal vs. post-ductal saturation difference >10%). - This is a **pathognomonic finding** in PPHN and helps differentiate it from cyanotic congenital heart disease. *Incorrect: Severe coarctation* - **Coarctation of the aorta** causes obstruction to blood flow, leading to **blood pressure differences** and **weak femoral pulses**, but does NOT primarily cause differential cyanosis. - While it may be associated with differential saturation in rare cases with severe pre-ductal coarctation and right-to-left ductal shunting, this is **not the typical presentation**. - The hallmark findings are **hypertension in upper extremities** and **hypotension in lower extremities**, not differential oxygenation. *Incorrect: Interrupted aortic arch* - While interrupted aortic arch relies on a **PDA for lower body perfusion**, the blood through the ductus is typically **well-oxygenated** unless there are associated complex cardiac defects causing desaturation. - The typical presentation is **poor perfusion and shock** rather than isolated differential cyanosis. - Differential cyanosis would only occur in specific anatomical variants with obligatory right-to-left shunting, which is not the standard presentation.
Question 9: A newborn has been brought with seizures refractory to treatment and a continuous bruit through the anterior fontanelle. CT shows midline lesion with hypoechogenicity and dilated lateral ventricles. Most probable diagnosis is?
- A. Teratoma
- B. Vein of Galen malformation (Correct Answer)
- C. Encephalocele
- D. Arachnoid cyst
Explanation: ***Vein of Galen malformation*** - A **continuous bruit** over the anterior fontanelle in a newborn is a classic sign of a **Vein of Galen malformation**, indicating high blood flow through an arteriovenous shunt. - The imaging findings of a **midline hypoechoic lesion** (dilated venous structure) and **dilated lateral ventricles** (due to hydrocephalus from venous congestion or obstruction) are consistent with this diagnosis. *Teratoma* - While teratomas are **germ cell tumors** that can occur in the brain, they typically present as a more solid or mixed solid-cystic mass on imaging, not primarily as hypoechoic. - A teratoma would not typically produce a **continuous bruit** over the fontanelle. *Encephalocele* - An encephalocele is a **neural tube defect** involving protrusion of brain tissue and meninges through a skull defect, which would be evident on physical examination and imaging as a sac-like protrusion. - It does not present with a **continuous bruit** or an intracranial hypoechoic midline lesion like a vascular malformation. *Arachnoid cyst* - An arachnoid cyst is a **benign, fluid-filled sac** that typically appears as a well-demarcated, anechoic (on ultrasound) or CSF-attenuated lesion on CT, often displacing surrounding brain tissue. - It would not produce a **continuous bruit** and is not usually associated with the specific constellation of symptoms seen here.
Question 10: A dense persistent nephrogram may be seen in all of the following except:
- A. Severe hydronephrosis
- B. Acute ureteral obstruction
- C. Systemic hypertension (Correct Answer)
- D. Dehydration
Explanation: ***Systemic hypertension*** - **Systemic hypertension** itself does not directly cause diffuse prolongation of the nephrogram phase unless there's an associated acute kidney injury from a different cause. - While chronic hypertension can lead to **nephrosclerosis**, which may affect contrast excretion, a persistent, dense nephrogram isn't its typical acute imaging finding. *Severe hydronephrosis* - **Severe hydronephrosis**, reflecting chronic or acute obstruction, can lead to impaired glomerular filtration and tubular function, causing delayed and prolonged contrast excretion. - The contrast remains in the dilated tubules and collecting system for an extended period, resulting in a **dense persistent nephrogram**. *Acute ureteral obstruction* - **Acute ureteral obstruction** causes increased collecting system pressure, leading to decreased glomerular filtration rate and impaired tubular flow. - This results in the contrast medium pooling in the renal tubules and interstitium, leading to a **delayed and persistent nephrogram**. *Dehydration* - **Dehydration** can lead to altered renal hemodynamics and concentrated urine, which can prolong the nephrogram phase due to slower contrast clearance. - Reduced renal blood flow and increased reabsorption of water lead to a more concentrated and thus **denser nephrogram** that persists longer than usual.
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