A 21-year-old Cambodian patient with a history of rheumatic heart disease presents to his primary care physician for a routine check-up. He reports being compliant with monthly penicillin G injections since being diagnosed with rheumatic fever at age 15. He denies any major side effects from the treatment, except for the inconvenience of organizing transportation to a physician's office every month. On exam, the patient is found to have a loud first heart sound and a mid-diastolic rumble that is best heard at the apex. Which of the following is the next best step?

Continue intramuscular penicillin therapy

Switch to intramuscular cefotaxime, which has fewer side effects

Stop penicillin therapy in 4 years

Decrease frequency of injections to bimonthly

A 7-year-old boy is brought by his parents to his pediatrician due to progressive fatigue and shortness of breath while playing sports. He is otherwise healthy with no known medical disorders and no other symptoms. The boy was born at 39 weeks gestation via spontaneous vaginal delivery. He is up to date on all vaccines and is meeting all developmental milestones. On physical examination, his temperature is 36.9ºC (98.4ºF), pulse rate is 90/min, blood pressure is 100/70 mm Hg, and respiratory rate is 18/min. Pulses in all four extremities are equal and normally palpated; there is no radio-femoral delay. The pediatrician suspects a congenital heart disease after auscultation of the heart. Which of the following congenital heart diseases is most likely to present with the clinical features listed above?

Double-outlet right ventricle with subaortic ventricular septal defect

Complete atrioventricular septal defect

A 3-year-old boy is brought to the physician because of recurrent nosebleeds and fatigue for the past 2 months. He also frequently complains his head hurts. The patient has met all motoric milestones for his age but does not like to run because his legs start to hurt if he does. He is at the 40th percentile for both height and weight. His temperature is 37.0°C (98.6°F), pulse is 125/min, respirations are 32/min, and blood pressure in the right arm is 130/85 mm Hg. A grade 2/6 systolic murmur is heard in the left paravertebral region. Further evaluation of this patient is most likely to show which of the following findings?

Delayed pulse in lower extremities

Interarm difference in tissue oxygenation

A 68-year-old man is brought to the emergency department because of right-sided weakness for 2 hours. He has hypertension, dyslipidemia, and type 2 diabetes. Current medications include hydrochlorothiazide, metoprolol, amlodipine, pravastatin, and metformin. His pulse is 87/min and blood pressure is 164/98 mm Hg. Neurological examination shows right-sided weakness, facial droop, and hyperreflexia. Sensation is intact. Which of the following is the most likely cause of these findings?

Lipohyalinosis of penetrating vessels

Dissection of the vertebral artery

Stenosis of the internal carotid artery

Rupture of an intracranial aneurysm

A 14-year-old boy is brought to the emergency department from school after falling in gym class. He was unable to stand after the accident and has a painful and swollen knee. On presentation he says that he has never had an incident like this before; however, he does suffer from hard to control nosebleeds and prolonged bleeding after minor cuts. Based on his presentation a panel of bleeding tests is obtained with the following results: Bleeding time: Prolonged Prothrombin time: Normal Partial thromboplastin time: Prolonged Which of the following describes the function of the component that is defective in the most likely cause of this patient's symptoms?

Binds to subendothelial collagen

Binds to a nucleotide derivative

It is a cofactor for an epoxide reductase

Catalyzes the conversion of factor X

An 80-year-old man presents to the emergency department because of gnawing substernal chest pain that started an hour ago and radiates to his neck and left jaw. A 12-lead ECG is obtained and shows ST-segment elevation with newly developing Q waves. He is admitted for treatment. 4 days after hospitalization he suddenly develops altered mental status, and his blood pressure falls from 115/75 mm Hg to 80/40 mm Hg. Physical examination shows jugular venous distention, pulsus paradoxus, and distant heart sounds. What is the most likely cause of this patient's condition?

Compression of heart chambers by blood in the pericardial space

Arrhythmia caused by ventricular fibrillation

Acute pulmonary edema from left heart failure

Adult congenital heart disease for USMLE Step 2 CK: High-Yield Internal Medicine Lessons

Acyanotic Defects - Shunt Shenanigans

Left-to-right shunts that increase pulmonary blood flow. Initially acyanotic, but can lead to pulmonary hypertension.

Defect	Epidemiology	Murmur	Classic CXR/ECG	Treatment
ASD	Ostium secundum most common	Wide, fixed split S2; systolic ejection murmur at upper left sternal border	RA/RV enlargement	Percutaneous device closure
VSD	Most common congenital heart defect	📌 'Holosystolic, Harsh-sounding' murmur at lower left sternal border	LA/LV enlargement	Surgical repair for large defects
PDA	Associated with congenital rubella	Continuous, machine-like murmur	Normal or LVH	Indomethacin (to close); Prostaglandins (to keep open)

⭐ Eisenmenger Syndrome: A severe, late complication where the initial left-to-right shunt reverses to a right-to-left shunt due to progressive pulmonary hypertension, causing late-onset cyanosis.

Cyanotic Defects - Blue Blood Blues

Tetralogy of Fallot (TOF): Most common cyanotic defect. 📌 Mnemonic: PROVe
- Pulmonary stenosis (determines severity)
- Right ventricular hypertrophy (RVH)
- Overriding aorta
- Ventricular septal defect (VSD)
"Tet Spells": Paroxysms of cyanosis, often during crying or feeding, from ↑ right-to-left shunting.
- Management: Knee-chest position (↑ SVR), O₂, morphine.
Eisenmenger Syndrome: An uncorrected L-to-R shunt (e.g., VSD, ASD) leads to severe pulmonary hypertension. The shunt reverses to R-to-L, causing late-onset cyanosis, clubbing, and polycythemia.

⭐ Exam Favorite: The classic chest X-ray finding for TOF is a "boot-shaped heart" due to RVH and an upturned cardiac apex.

Tetralogy of Fallot: Boot-shaped heart on chest X-ray

Obstructive Lesions - No-Flow Fiascos

Coarctation of the Aorta (CoA):
- BP discrepancy: ↑ upper extremities, ↓ lower extremities.
- Weak, delayed femoral pulses.
- Associated with Turner Syndrome & bicuspid aortic valve.
- 📌 Mnemonic: "3" sign on CXR from aortic indentation.

Chest X-ray: Coarctation of Aorta with Rib Notching

⭐ Rib notching on CXR is a classic sign, caused by collateral flow through enlarged intercostal arteries.

Valvular Stenosis:
- Aortic Stenosis (AS): Systolic ejection murmur, often a bicuspid valve. Can cause angina, syncope, dyspnea (SAD).
- Pulmonic Stenosis (PS): Systolic ejection click, RVH. Associated with Noonan Syndrome.

ACHD Management - Heartfelt Hurdles

Infective Endocarditis (IE) Prophylaxis: For high-risk patients (prosthetic valves, prior IE, specific unrepaired/repaired CHD) before dental/respiratory procedures. Amoxicillin 2g PO is standard.
Anticoagulation: Essential for mechanical valves, Fontan circulation, and managing atrial fibrillation (AF) risk.
Arrhythmia Risk: AF/atrial flutter are common, elevating stroke risk. Requires rate/rhythm control & anticoagulation.
Pregnancy Considerations: High-risk endeavor requiring a specialized cardio-obstetrics team. Eisenmenger syndrome is an absolute contraindication.

⭐ In repaired Tetralogy of Fallot, the leading cause of late mortality is sudden cardiac death from ventricular arrhythmias.

Bicuspid aortic valve is the most common adult congenital heart disease, leading to early aortic stenosis/regurgitation.

An atrial septal defect (ASD) classically presents with a wide, fixed split S2 and can cause paradoxical emboli.

Coarctation of the aorta is linked to Turner syndrome, upper extremity hypertension, and rib notching on chest X-ray.

Eisenmenger syndrome is the reversal of a left-to-right shunt from chronic pulmonary hypertension, causing late-onset cyanosis.

Tetralogy of Fallot is the most common cyanotic defect; look for "tet spells" relieved by squatting.

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