A 75-year-old man with hypercholesterolemia, coronary artery disease, and history of a transient ischemic attack (TIA) comes to your office for evaluation of hypertension. Previously, his blood pressure was controlled with diet and an ACE inhibitor. Today, his blood pressure is 180/115 mm Hg, and his creatinine is increased from 0.54 to 1.2 mg/dL. The patient reports that he has been compliant with his diet and blood pressure medications. What is the most likely cause of his hypertension?
Q172
A 34-year-old man presents to the emergency department with a headache that has lasted for 2 hours. His headache is severe and he rates it as a 10/10 on the pain scale. It is generalized and associated with nausea and photophobia. He denies any history of head trauma or fever. He has a history of migraines, but he says this headache is worse than any he has had before. He has no other significant past medical history and takes no medications. His father has chronic kidney disease. Physical examination reveals: blood pressure 125/66 mm Hg, heart rate 80/min, and temperature 37.2°C (99.0°F). The patient is awake, alert, and oriented, but he is in severe distress due to the pain. On physical examination, his neck is stiff with flexion. Motor strength is 5/5 in all 4 limbs and sensation is intact. Fundoscopic examination results are within normal limits. What is the next best step in the management of this patient?
Q173
A 70-year-old man with hypertension and type 2 diabetes mellitus is admitted to the hospital 8 hours after the onset of impaired speech and right-sided weakness. Two days after admission, he becomes confused and is difficult to arouse. His pulse is 64/min and blood pressure is 166/96 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 95%. Fundoscopic examination shows bilateral optic disc swelling. He is intubated and mechanically ventilated. A CT scan of the brain shows hypoattenuation in the territory of the left middle cerebral artery with surrounding edema and a 1-cm midline shift to the right. Which of the following interventions is most likely to result in a decrease in this patient's intracranial pressure?
Q174
A 39-year-old woman with a history of migraine headaches is brought to the emergency room because of a severe, sudden-onset, throbbing headache and double vision for 1 hour. She says that she has been having frequent headaches and has not had her period in several months. Her blood pressure is 93/61 mm Hg. Visual field testing shows decreased visual acuity and bilateral temporal visual field defects. Which of the following is the most likely diagnosis?
Q175
A 67-year-old male presents to his primary care physician complaining of increased fatigue over the last year. He also says that his friends say he appears to be more pale. His past medical history is significant for 10 years of arthritis. Physical exam reveals conjunctival pallor. Based on clinical suspicion RBC tests are ordered showing a mean corpuscular volume (MCV) of 75 fl (normal 80-100 fl) and a peripheral blood smear is obtained and found to be normal. Iron studies show a serum iron of 30 micromolar (normal range 50-170) and a serum ferritin of 300 micrograms/liter (normal range 15-200). What is the most likely diagnosis in this patient?
Q176
A 55-year-old patient is brought to the emergency department because he has had sharp chest pain for the past 3 hours. He reports that he can only take shallow breaths because deep inspiration worsens the pain. He also reports that the pain increases with coughing. Two weeks ago, he underwent cardiac catheterization for an acute myocardial infarction. Current medications include aspirin, ticagrelor, atorvastatin, metoprolol, and lisinopril. His temperature is 38.54°C (101.1°F), pulse is 55/min, respirations are 23/min, and blood pressure is 125/75 mm Hg. Cardiac examination shows a high-pitched scratching sound best heard when the patient is sitting upright and during expiration. An ECG shows diffuse ST elevations and ST depression in aVR and V1. An echocardiography shows no abnormalities. Which of the following is the most appropriate treatment in this patient?
Q177
A 69-year-old woman presents to her physician’s office with cough, increasing fatigue, and reports an alarming loss of 15 kg (33 lb) weight over the last 4 months. She says that she has observed this fatigue and cough to be present over the past year, but pushed it aside citing her age as a reason. The cough has been progressing and the weight loss is really worrying her. She also observed blood-tinged sputum twice over the last week. Past medical history is noncontributory. She does not smoke and does not use recreational drugs. She is relatively active and follows a healthy diet. Today, her vitals are normal. On examination, she appears frail and pale. At auscultation, her lung has a slight expiratory wheeze. A chest X-ray shows a coin-shaped lesion in the periphery of the middle lobe of the right lung. The nodule is biopsied by interventional radiology (see image). Which of the following types of cancer is most likely associated with this patient’s symptoms?
Q178
A 30-year-old man with Down syndrome is brought to the physician by his mother for the evaluation of fatigue. Physical examination shows bluish-colored lips and digital clubbing that were not present at his most recent examination. Right heart catheterization shows a right atrial pressure of 32 mmHg. Which of the following is most likely involved in the pathogenesis of this patient's current condition?
Q179
A 35-year-old woman presents to the ER with shortness of breath, cough, and severe lower limb enlargement. The dyspnea was of sudden onset, started a week ago, and increased with exercise but did not disappear with rest. Her cough was dry, persistent, and non-productive. She has a family history of maternal hypertension. Her vital signs include heart rate 106/min, respiratory rate 28/min, and blood pressure 140/90 mm Hg. On physical examination, thoracic expansion was diminished on the right side with rhonchi and crackles on the lower two-thirds of both sides, with left predominance. A systolic murmur was heard on the tricuspid foci, which increased in intensity with inspiration. There was jugular engorgement when the bed was placed at 50°. Palpation of the abdomen was painful on the right hypochondrium, with hepatomegaly 4 cm below the lower costal edge. Hepatojugular reflux was present. Soft, painless, pitting edema was present in both lower limbs up until the middle third of both legs. Lung computed tomography (CT) and transthoracic echocardiogram were performed and detected right heart failure and severe pulmonary fibrosis. What is the most likely diagnosis?
Q180
A 33-year-old man presents to the emergency department with dizziness. He states he has experienced a sustained sense of disequilibrium for the past 2 days. He feels that the floor is unstable/moving. The patient is otherwise healthy and does not have any other medical diagnoses. The patient is currently taking vitamin C as multiple family members are currently ill and he does not want to get sick. His temperature is 98.1°F (36.7°C), blood pressure is 120/83 mmHg, pulse is 73/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam is notable for a horizontal nystagmus. The Dix-Hallpike maneuver does not provoke symptoms and examination of the patient’s cranial nerves is unremarkable. Which of the following is the most likely diagnosis?
Cardiology US Medical PG Practice Questions and MCQs
Question 171: A 75-year-old man with hypercholesterolemia, coronary artery disease, and history of a transient ischemic attack (TIA) comes to your office for evaluation of hypertension. Previously, his blood pressure was controlled with diet and an ACE inhibitor. Today, his blood pressure is 180/115 mm Hg, and his creatinine is increased from 0.54 to 1.2 mg/dL. The patient reports that he has been compliant with his diet and blood pressure medications. What is the most likely cause of his hypertension?
A. Pheochromocytoma
B. Coarctation of the aorta
C. Renal artery stenosis (Correct Answer)
D. Progression of his essential hypertension
E. Hypothyroidism
Explanation: **Renal artery stenosis**
- The sudden, severe elevation in blood pressure, coupled with a significant increase in **creatinine** while on an **ACE inhibitor**, strongly suggests **renal artery stenosis**.
- ACE inhibitors can cause **acute kidney injury** in patients with bilateral renal artery stenosis (or stenosis of a solitary functioning kidney) by dilating the efferent arteriole, leading to a decrease in **glomerular filtration pressure**.
*Pheochromocytoma*
- While pheochromocytoma can cause severe hypertension, it's typically associated with paroxysmal episodes, **palpitations**, **headaches**, and **sweating**, which are not reported here.
- It would not typically explain the **acute renal dysfunction** seen with ACE inhibitor use.
*Coarctation of the aorta*
- Most commonly diagnosed in **childhood or early adulthood** and presents with a blood pressure difference between the upper and lower extremities.
- It is unlikely to present for the first time in a 75-year-old with this specific clinical picture of **renal dysfunction secondary to ACE inhibitor use**.
*Progression of his essential hypertension*
- While essential hypertension can worsen, the **acute and severe increase** in blood pressure along with the substantial rise in **creatinine** strongly points to a **secondary cause**, especially in the context of ACE inhibitor use.
- Simple progression would not typically cause such a dramatic and acute change in **creatinine levels**.
*Hypothyroidism*
- Hypothyroidism can contribute to **diastolic hypertension** and increased cardiovascular risk, but it does not typically cause a **sudden, severe hypertensive crisis** or **acute kidney injury** in response to ACE inhibitors.
- Other symptoms of hypothyroidism (**fatigue**, **weight gain**, **cold intolerance**) are not mentioned.
Question 172: A 34-year-old man presents to the emergency department with a headache that has lasted for 2 hours. His headache is severe and he rates it as a 10/10 on the pain scale. It is generalized and associated with nausea and photophobia. He denies any history of head trauma or fever. He has a history of migraines, but he says this headache is worse than any he has had before. He has no other significant past medical history and takes no medications. His father has chronic kidney disease. Physical examination reveals: blood pressure 125/66 mm Hg, heart rate 80/min, and temperature 37.2°C (99.0°F). The patient is awake, alert, and oriented, but he is in severe distress due to the pain. On physical examination, his neck is stiff with flexion. Motor strength is 5/5 in all 4 limbs and sensation is intact. Fundoscopic examination results are within normal limits. What is the next best step in the management of this patient?
A. Acetazolamide
B. CT head (Correct Answer)
C. Lumbar puncture
D. Sumatriptan
E. Antibiotics
Explanation: ***Correct: CT head***
- A **sudden, severe headache** ("worst headache of my life") along with **nuchal rigidity** (stiff neck) is highly concerning for a **subarachnoid hemorrhage (SAH)**.
- A non-contrast **CT head** is the immediate and most appropriate first diagnostic step to rule out acute intracranial bleeding.
- CT scan has **high sensitivity (>95%) for SAH within the first 6 hours** and should always precede lumbar puncture to avoid risk of herniation.
*Incorrect: Acetazolamide*
- **Acetazolamide** is a carbonic anhydrase inhibitor used in conditions like **idiopathic intracranial hypertension** or to reduce cerebrospinal fluid production.
- It is not indicated for the acute management of a sudden-onset severe headache with signs of meningeal irritation.
*Incorrect: Lumbar puncture*
- While a **lumbar puncture** (LP) can diagnose SAH if the CT is negative and suspicion remains high (xanthochromia in CSF), it should only be performed **after a CT scan** has ruled out mass effect or hydrocephalus.
- Performing an LP before a CT scan in the presence of increased intracranial pressure could lead to **herniation**.
*Incorrect: Sumatriptan*
- **Sumatriptan** is a triptan medication used for the abortive treatment of **migraine headaches**.
- This patient's headache is described as "worse than any he has had before" and is accompanied by nuchal rigidity, making it atypical for a routine migraine and warranting investigation for a secondary cause.
*Incorrect: Antibiotics*
- **Antibiotics** would be considered if there was strong evidence of bacterial meningitis (e.g., fever, rash, altered mental status, and CSF findings consistent with bacterial infection).
- While nuchal rigidity is seen in meningitis, the absence of fever and the sudden onset suggest SAH as a primary concern, and antibiotics would not be the initial step without further investigation.
Question 173: A 70-year-old man with hypertension and type 2 diabetes mellitus is admitted to the hospital 8 hours after the onset of impaired speech and right-sided weakness. Two days after admission, he becomes confused and is difficult to arouse. His pulse is 64/min and blood pressure is 166/96 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 95%. Fundoscopic examination shows bilateral optic disc swelling. He is intubated and mechanically ventilated. A CT scan of the brain shows hypoattenuation in the territory of the left middle cerebral artery with surrounding edema and a 1-cm midline shift to the right. Which of the following interventions is most likely to result in a decrease in this patient's intracranial pressure?
A. Decrease the blood pressure
B. Increase the fraction of inhaled oxygen
C. Decrease the heart rate
D. Increase the respiratory rate (Correct Answer)
E. Increase the positive end-expiratory pressure
Explanation: ***Increase the respiratory rate***
- Increasing the respiratory rate (and thus minute ventilation) leads to a decrease in the partial pressure of **arterial carbon dioxide (PaCO2)**.
- A lower PaCO2 causes **cerebral vasoconstriction**, which reduces cerebral blood volume and subsequently decreases intracranial pressure (ICP).
*Decrease the blood pressure*
- While uncontrolled hypertension is harmful, a drastic reduction in blood pressure could compromise **cerebral perfusion pressure (CPP)**, especially in the setting of elevated ICP.
- Maintaining an adequate CPP is crucial to prevent further brain ischemia.
*Increase the fraction of inhaled oxygen*
- The patient's oxygen saturation is already 95% on room air, indicating adequate oxygenation.
- Increasing the fraction of inspired oxygen (FiO2) would not directly reduce ICP and is not the primary intervention for this purpose, unless hypoxemia is present.
*Decrease the heart rate*
- The patient's pulse of 64/min is within a normal range; there's no indication that a high heart rate is contributing to elevated ICP.
- Bradycardia as an isolated intervention would not effectively lower ICP.
*Increase the positive end-expiratory pressure*
- **Positive end-expiratory pressure (PEEP)** can increase intrathoracic pressure, which may impede venous outflow from the brain, potentially *increasing* ICP.
- While PEEP is used for lung protection, high levels should be used cautiously in patients with elevated ICP.
Question 174: A 39-year-old woman with a history of migraine headaches is brought to the emergency room because of a severe, sudden-onset, throbbing headache and double vision for 1 hour. She says that she has been having frequent headaches and has not had her period in several months. Her blood pressure is 93/61 mm Hg. Visual field testing shows decreased visual acuity and bilateral temporal visual field defects. Which of the following is the most likely diagnosis?
A. Migraine with aura
B. Cluster headache
C. Pituitary apoplexy (Correct Answer)
D. Sheehan syndrome
E. Transient ischemic attack
Explanation: ***Pituitary apoplexy***
- The sudden onset of a **severe headache**, **double vision** (due to cranial nerve compression), **bitemporal hemianopsia** (loss of peripheral vision in both eyes due to optic chiasm compression), and **hypotension** in a patient with a history of headaches and amenorrhea (suggesting pituitary dysfunction) are classic signs of pituitary apoplexy.
- Pituitary apoplexy is an acute hemorrhage or infarction of the pituitary gland, often occurring in the setting of a pre-existing pituitary adenoma, which can cause both mass effect symptoms and endocrine dysfunction (e.g., amenorrhea from prolactinoma or hypogonadism).
*Migraine with aura*
- While the patient has a history of migraines, the **sudden onset**, associated **double vision** (cranial nerve palsy), and **bitemporal visual field deficits** are not typical features of a standard migraine with aura, which usually presents with transient neurological symptoms followed by headache.
- Migraines typically don't cause acute hormonal deficiencies leading to amenorrhea or the specific pattern of visual field loss seen here.
*Cluster headache*
- Cluster headaches are characterized by **severe, unilateral ocular or periorbital pain** accompanied by ipsilateral autonomic symptoms (e.g., ptosis, miosis, lacrimation, nasal congestion).
- They do not typically present with double vision, bitemporal hemianopsia, or signs of pituitary dysfunction like hypotension and amenorrhea.
*Sheehan syndrome*
- Sheehan syndrome results from **ischemic necrosis of the pituitary gland** due to massive postpartum hemorrhage, leading to chronic hypopituitarism.
- While it causes amenorrhea and other signs of hypopituitarism, it is a chronic condition and does not present with acute, sudden-onset severe headache, double vision, and bitemporal visual field defects like pituitary apoplexy.
*Transient ischemic attack*
- A TIA involves **transient neurological dysfunction caused by focal brain, spinal cord, or retinal ischemia** without acute infarction.
- While it can cause transient visual disturbances or weakness, it would not explain the severe headache, bitemporal visual field loss pattern, double vision due to cranial nerve compression, or the patient's history of amenorrhea related to pituitary dysfunction.
Question 175: A 67-year-old male presents to his primary care physician complaining of increased fatigue over the last year. He also says that his friends say he appears to be more pale. His past medical history is significant for 10 years of arthritis. Physical exam reveals conjunctival pallor. Based on clinical suspicion RBC tests are ordered showing a mean corpuscular volume (MCV) of 75 fl (normal 80-100 fl) and a peripheral blood smear is obtained and found to be normal. Iron studies show a serum iron of 30 micromolar (normal range 50-170) and a serum ferritin of 300 micrograms/liter (normal range 15-200). What is the most likely diagnosis in this patient?
A. Lead poisoning anemia
B. Alpha-thalassemia
C. Iron deficiency anemia
D. Anemia of chronic disease (Correct Answer)
E. Beta-thalassemia
Explanation: ***Anemia of chronic disease***
- This patient's **microcytic anemia** (MCV 75 fL) with **chronic inflammation** (10 years of arthritis), **low serum iron** (30 µmol), and **elevated ferritin** (300 µg/L) is classic for anemia of chronic disease.
- The **elevated ferritin** is the key finding that distinguishes this from iron deficiency anemia. In anemia of chronic disease, inflammatory cytokines stimulate **hepcidin production**, which blocks iron release from macrophages and intestinal absorption, creating a functional iron deficiency despite adequate body iron stores (reflected by high ferritin).
- Chronic inflammatory conditions like rheumatoid arthritis commonly cause this type of anemia through IL-6 mediated hepcidin upregulation.
*Iron deficiency anemia*
- Although iron deficiency causes **microcytic anemia** and **low serum iron**, it is characterized by **low ferritin** (typically <15 µg/L), not elevated ferritin as seen in this patient.
- The **elevated ferritin (300 µg/L)** definitively rules out iron deficiency anemia, as ferritin levels reflect body iron stores and would be depleted in true iron deficiency.
- Both conditions can present with fatigue and pallor, but the iron studies clearly differentiate them.
*Lead poisoning anemia*
- Lead poisoning causes **microcytic anemia** through inhibition of heme synthesis, but the **peripheral blood smear would show basophilic stippling**, which is specifically noted as normal in this case.
- There is no history of occupational exposure, old housing, or other risk factors for lead poisoning.
- Iron studies in lead poisoning typically show elevated or normal ferritin with increased iron stores, not the specific pattern seen in anemia of chronic disease.
*Alpha-thalassemia*
- Alpha-thalassemia causes **microcytic anemia** but is a **hereditary hemoglobinopathy** that typically presents earlier in life with lifelong mild anemia.
- New-onset symptoms at age 67 with progressive fatigue over one year argues against a congenital disorder.
- Iron studies in thalassemia show **normal or elevated ferritin** and normal-to-high serum iron, not the low serum iron seen here due to inflammation.
*Beta-thalassemia*
- Like alpha-thalassemia, beta-thalassemia is a **genetic disorder** typically diagnosed in childhood or early adulthood, making new diagnosis at age 67 unlikely.
- Iron studies would show **normal or elevated iron stores** (often with secondary hemochromatosis in transfusion-dependent cases), not the pattern of functional iron deficiency seen with chronic inflammation.
- The presence of chronic arthritis and the specific iron study pattern strongly favor an acquired inflammatory anemia rather than a hereditary hemoglobinopathy.
Question 176: A 55-year-old patient is brought to the emergency department because he has had sharp chest pain for the past 3 hours. He reports that he can only take shallow breaths because deep inspiration worsens the pain. He also reports that the pain increases with coughing. Two weeks ago, he underwent cardiac catheterization for an acute myocardial infarction. Current medications include aspirin, ticagrelor, atorvastatin, metoprolol, and lisinopril. His temperature is 38.54°C (101.1°F), pulse is 55/min, respirations are 23/min, and blood pressure is 125/75 mm Hg. Cardiac examination shows a high-pitched scratching sound best heard when the patient is sitting upright and during expiration. An ECG shows diffuse ST elevations and ST depression in aVR and V1. An echocardiography shows no abnormalities. Which of the following is the most appropriate treatment in this patient?
A. Perform pericardiocentesis
B. Increase aspirin dose (Correct Answer)
C. Administer nitroglycerin
D. Start heparin infusion
E. Perform CT angiography
Explanation: ***Increase aspirin dose***
- This patient presents with symptoms and signs consistent with **post-myocardial infarction pericarditis**. The classic treatment involves high-dose aspirin (750-1000 mg three times daily).
- The patient's cardiac medications, including current low-dose aspirin (for antiplatelet effect), do not adequately address the inflammation associated with pericarditis. Increasing the dose provides the necessary anti-inflammatory effect.
- This occurs within days to weeks after MI due to transmural myocardial inflammation. NSAIDs are often avoided post-MI due to concerns about impaired ventricular healing, making high-dose aspirin the preferred anti-inflammatory agent.
*Perform pericardiocentesis*
- **Pericardiocentesis** is indicated for **cardiac tamponade** or large, symptomatic pericardial effusions causing hemodynamic compromise.
- The echocardiography in this patient shows no abnormalities, ruling out significant pericardial effusion or tamponade.
*Administer nitroglycerin*
- **Nitroglycerin** is used to relieve **angina** by causing vasodilation and reducing myocardial oxygen demand.
- The patient's chest pain, diffuse ST elevations, and friction rub are indicative of **pericarditis**, not ongoing myocardial ischemia, making nitroglycerin inappropriate.
*Start heparin infusion*
- **Heparin** is an anticoagulant used to prevent thrombus formation, often in conditions like **deep vein thrombosis**, **pulmonary embolism**, or in acute coronary syndromes to prevent clot propagation.
- There is no indication of active thrombus formation or worsening acute coronary syndrome that would warrant heparin infusion. Moreover, anticoagulation is **contraindicated in pericarditis** as it increases the risk of hemorrhagic pericardial effusion.
*Perform CT angiography*
- **CT angiography** is primarily used to diagnose **pulmonary embolism** or **aortic dissection**.
- The clinical presentation, including the diffuse ST elevations, pericardial friction rub, and recent MI, strongly points to pericarditis, making CT angiography an unnecessary investigation in this context.
Question 177: A 69-year-old woman presents to her physician’s office with cough, increasing fatigue, and reports an alarming loss of 15 kg (33 lb) weight over the last 4 months. She says that she has observed this fatigue and cough to be present over the past year, but pushed it aside citing her age as a reason. The cough has been progressing and the weight loss is really worrying her. She also observed blood-tinged sputum twice over the last week. Past medical history is noncontributory. She does not smoke and does not use recreational drugs. She is relatively active and follows a healthy diet. Today, her vitals are normal. On examination, she appears frail and pale. At auscultation, her lung has a slight expiratory wheeze. A chest X-ray shows a coin-shaped lesion in the periphery of the middle lobe of the right lung. The nodule is biopsied by interventional radiology (see image). Which of the following types of cancer is most likely associated with this patient’s symptoms?
A. Small cell carcinoma
B. Large cell carcinoma
C. Mesothelioma
D. Adenocarcinoma (Correct Answer)
E. Squamous cell carcinoma
Explanation: ***Adenocarcinoma***
- **Adenocarcinoma** is the most common type of lung cancer in non-smokers and often presents as a **peripheral solitary nodule or mass** on chest imaging.
- The patient's symptoms of **cough, fatigue, significant weight loss, and hemoptysis** are classic for lung malignancy. The coin-shaped lesion in the periphery is highly suggestive of adenocarcinoma.
*Small cell carcinoma*
- **Small cell carcinoma** is strongly associated with **smoking** and typically presents as a **central mass** with early metastasis.
- While it can cause similar systemic symptoms, the patient's non-smoking history and peripheral lesion make it less likely.
*Large cell carcinoma*
- **Large cell carcinoma** is a diagnosis of exclusion and often presents as a large, **peripheral mass**, but is less common than adenocarcinoma in non-smokers.
- Its incidence is higher in smokers, and it lacks the specific glandular differentiation seen in adenocarcinoma.
*Mesothelioma*
- **Mesothelioma** is a rare cancer of the pleura, strongly linked to **asbestos exposure**, which is not present in this patient's history.
- It typically manifests with **pleural effusions** and thickening, rather than a solitary coin-shaped lesion within the lung parenchyma.
*Squamous cell carcinoma*
- **Squamous cell carcinoma** is highly associated with **smoking** and typically presents as a **central mass** with cavitation.
- The patient's non-smoking history and the peripheral location of the lesion make this diagnosis less probable.
Question 178: A 30-year-old man with Down syndrome is brought to the physician by his mother for the evaluation of fatigue. Physical examination shows bluish-colored lips and digital clubbing that were not present at his most recent examination. Right heart catheterization shows a right atrial pressure of 32 mmHg. Which of the following is most likely involved in the pathogenesis of this patient's current condition?
A. Aortic valve regurgitation
B. Reversible pulmonary hypertension
C. Reversed intracardiac shunting (Correct Answer)
D. Asymmetric septal hypertrophy
E. Right ventricular hypertrophy
Explanation: ***Reversed intracardiac shunting***
- The patient's signs of **cyanosis** (bluish lips) and **digital clubbing**, in the context of Down syndrome, indicate **Eisenmenger syndrome** - a reversal of an intracardiac shunt from left-to-right to right-to-left.
- In Down syndrome, common congenital heart defects like **atrioventricular septal defect (AVSD)**, **ventricular septal defect (VSD)**, or **atrial septal defect (ASD)** initially cause left-to-right shunting with chronic pulmonary overcirculation.
- This leads to **irreversible pulmonary vascular remodeling** and **pulmonary hypertension**, eventually causing the shunt to reverse (right-to-left), resulting in **deoxygenated blood bypassing the lungs** and causing cyanosis and clubbing.
- The markedly elevated **right atrial pressure (32 mmHg)** reflects the severe pulmonary hypertension and right heart strain.
*Aortic valve regurgitation*
- **Aortic valve regurgitation** primarily causes **left ventricular volume overload** and symptoms of left-sided heart failure (e.g., dyspnea on exertion, bounding pulses), not typically cyanosis or digital clubbing.
- While chronic severe aortic regurgitation can eventually lead to pulmonary hypertension, it would not cause right-to-left shunting or the degree of right atrial pressure elevation seen here.
*Reversible pulmonary hypertension*
- The presence of **cyanosis** and **digital clubbing** indicates **Eisenmenger syndrome** with **irreversible** pulmonary vascular disease where the shunt has permanently reversed from left-to-right to right-to-left.
- **Reversible pulmonary hypertension** would not yet have progressed to such prominent signs of chronic hypoxemia like clubbing, and interventions could still reduce pulmonary pressures before shunt reversal occurs.
*Asymmetric septal hypertrophy*
- **Asymmetric septal hypertrophy** is characteristic of **hypertrophic cardiomyopathy**, which causes **left ventricular outflow tract obstruction** and symptoms like syncope, chest pain, and dyspnea.
- It does not explain the development of cyanosis, digital clubbing, or the markedly elevated right atrial pressures seen with reversed intracardiac shunting.
*Right ventricular hypertrophy*
- While **right ventricular hypertrophy** is a consequence of chronic pulmonary hypertension and elevated right atrial pressure, it is a **compensatory structural change** rather than the primary pathogenetic mechanism causing cyanosis.
- The underlying cause of the cyanotic condition is the **reversed intracardiac shunt** (right-to-left) due to severe pulmonary hypertension overwhelming the left-sided pressures.
Question 179: A 35-year-old woman presents to the ER with shortness of breath, cough, and severe lower limb enlargement. The dyspnea was of sudden onset, started a week ago, and increased with exercise but did not disappear with rest. Her cough was dry, persistent, and non-productive. She has a family history of maternal hypertension. Her vital signs include heart rate 106/min, respiratory rate 28/min, and blood pressure 140/90 mm Hg. On physical examination, thoracic expansion was diminished on the right side with rhonchi and crackles on the lower two-thirds of both sides, with left predominance. A systolic murmur was heard on the tricuspid foci, which increased in intensity with inspiration. There was jugular engorgement when the bed was placed at 50°. Palpation of the abdomen was painful on the right hypochondrium, with hepatomegaly 4 cm below the lower costal edge. Hepatojugular reflux was present. Soft, painless, pitting edema was present in both lower limbs up until the middle third of both legs. Lung computed tomography (CT) and transthoracic echocardiogram were performed and detected right heart failure and severe pulmonary fibrosis. What is the most likely diagnosis?
A. Left-sided heart failure
B. Liver disease
C. Cor pulmonale (Correct Answer)
D. Budd-Chiari syndrome
E. Coronary artery disease
Explanation: ***Cor pulmonale***
- The combination of **shortness of breath**, **dependent edema**, **jugular venous distension**, **hepatomegaly** with **hepatojugular reflux**, and a **right-sided systolic murmur** that increases with inspiration are all classic signs of **right-sided heart failure**.
- The presence of **severe pulmonary fibrosis** identified on CT supports the diagnosis, as chronic lung disease is a common cause of **pulmonary hypertension** leading to **right ventricular hypertrophy** and failure, known as cor pulmonale.
*Left-sided heart failure*
- While **dyspnea** and **cough** are present, there is a clear predominance of signs pointing to **right-sided heart failure**, such as pronounced **peripheral edema**, **JVD**, and **hepatomegaly**.
- **Left-sided heart failure** typically presents with pulmonary congestion (e.g., pulmonary edema, Orthopnea, paroxysmal nocturnal dyspnea) and would not explain the prominent signs of fluid overload in the systemic circulation.
*Liver disease*
- **Hepatomegaly** and **edema** can be present in liver disease, but the patient also exhibits significant **dyspnea**, **cough**, and **right-sided cardiac findings** (e.g., tricuspid murmur, JVD, right-sided heart failure on echo).
- The primary symptoms and diagnostic findings point towards a **cardiopulmonary etiology** rather than primary liver pathology.
*Budd-Chiari syndrome*
- This syndrome involves **hepatic venous outflow obstruction**, leading to **hepatomegaly**, **abdominal pain**, and **ascites**. While **hepatomegaly** and abdominal pain are present, the overall clinical picture with **severe pulmonary fibrosis** and clear signs of **right heart failure** is more consistent with cor pulmonale.
- **Budd-Chiari syndrome** typically does not present with primary respiratory symptoms or right-sided heart murmurs secondary to pulmonary issues.
*Coronary artery disease*
- **Coronary artery disease** primarily affects the left ventricle and would lead to symptoms of **left-sided heart failure** (e.g., angina, shortness of breath on exertion due to pulmonary congestion).
- The patient's presentation is overwhelmingly indicative of **right-sided heart failure**, with pulmonary fibrosis as the likely underlying cause, rather than ischemic heart disease.
Question 180: A 33-year-old man presents to the emergency department with dizziness. He states he has experienced a sustained sense of disequilibrium for the past 2 days. He feels that the floor is unstable/moving. The patient is otherwise healthy and does not have any other medical diagnoses. The patient is currently taking vitamin C as multiple family members are currently ill and he does not want to get sick. His temperature is 98.1°F (36.7°C), blood pressure is 120/83 mmHg, pulse is 73/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam is notable for a horizontal nystagmus. The Dix-Hallpike maneuver does not provoke symptoms and examination of the patient’s cranial nerves is unremarkable. Which of the following is the most likely diagnosis?
A. Vertebrobasilar stroke
B. Benign paroxysmal positional vertigo
C. Labyrinthitis
D. Meniere disease
E. Vestibular neuritis (Correct Answer)
Explanation: ***Vestibular neuritis***
- The patient's **acute onset of sustained dizziness, horizontal nystagmus**, and otherwise healthy status points to a peripheral vestibular cause, with **vestibular neuritis** being highly likely in the absence of hearing loss.
- The **normal Dix-Hallpike maneuver** and lack of positional vertigo rule out BPPV, while the absence of other neurological deficits argues against a central cause like stroke.
*Vertebrobasilar stroke*
- A vertebrobasilar stroke would typically present with additional **neurological deficits**, such as cranial nerve palsies, ataxia, or motor weakness, which are absent here.
- While a stroke can cause dizziness, the isolated **horizontal nystagmus** without other "HINTS" exam findings (Head Impulse, Nystagmus, Test of Skew) suggesting a central lesion makes this less likely.
*Benign paroxysmal positional vertigo*
- This condition is characterized by brief episodes of vertigo triggered by specific **head movements**, and would typically be provoked by a **Dix-Hallpike maneuver**.
- The patient's **sustained sense of disequilibrium** for two days, rather than brief positional vertigo, makes BPPV unlikely.
*Labyrinthitis*
- Labyrinthitis is an inflammation of the inner ear that causes **dizziness and hearing loss**, which is not mentioned in this case.
- While it shares some symptoms with vestibular neuritis (sustained dizziness), the **absence of hearing symptoms** helps differentiate it.
*Meniere disease*
- Meniere disease presents with a classic triad of **vertigo, tinnitus, and fluctuating hearing loss**, along with aural fullness.
- The patient's symptoms do not include tinnitus, hearing changes, or aural fullness, making Meniere disease an improbable diagnosis.