Study guide
1.11 Adult Congenital Heart Disease & Coarctation of the Aorta
ABIM Internal Medicine Exam
Track your progress as you read
Finished sections get a check mark.
1 Chapter 0: The ABIM Exam, Blueprint & Study Strategy
0.1 Exam Format, Question Types & the 2026 Blueprint 0.2 Eligibility, Registration, Fees & Exam-Day Logistics 0.3 Scoring, the Passing Standard & Score Reports 0.4 Building a Blueprint-Weighted Study Plan
2 Chapter 1: Cardiovascular Disease (14% of Exam)
1.1 Ischemic Heart Disease & Acute Coronary Syndromes 1.2 Antithrombotic Therapy, Secondary Prevention & Mechanical Complications of MI 1.3 Atrial Fibrillation, Atrial Flutter & Stroke Prevention 1.4 Supraventricular Tachycardias, Ventricular Arrhythmias & Bradycardias 1.5 Heart Failure: Classification, Staging & Guideline-Directed Medical Therapy 1.6 Device Therapy, HFpEF & the Cardiomyopathies 1.7 Valvular Heart Disease & Endocarditis Prophylaxis 1.8 Pericardial Disease & Acute Aortic Syndromes 1.9 Hypertension: Classification, Diagnosis & Secondary Causes 1.10 Hypertensive Crises & Dyslipidemia 1.11 Adult Congenital Heart Disease & Coarctation of the Aorta 1.12 Syncope: Reflex, Orthostatic & Cardiac Causes 1.13 Cardiac Physical Examination, Murmurs & Antithrombotic Selection
3 Chapter 2: Endocrinology, Diabetes & Metabolism (9% of Exam)
2.1 Diabetes Mellitus: Diagnosis, Glycemic Targets & Pharmacotherapy 2.2 Insulin Therapy, Hyperglycemic Emergencies & Chronic Complications 2.3 Thyroid Function Testing & Hypothyroidism 2.4 Thyrotoxicosis, Thyroid Storm & Thyroid Nodules 2.5 Adrenal & Pituitary Disorders 2.6 Hypercalcemia, Hypocalcemia & Disorders of Mineral Metabolism 2.7 Osteoporosis & Paget Disease of Bone 2.8 Ovarian, Testicular & Reproductive Endocrine Disorders 2.9 Hypoglycemia, Endocrine Tumors, Polyglandular Syndromes & Severe Hypertriglyceridemia
4 Chapter 3: Gastroenterology & Hepatology (9% of Exam)
3.1 Esophageal Disease 3.2 Stomach & Duodenal Disease and Gastrointestinal Hemorrhage 3.3 Inflammatory Bowel Disease 3.4 Celiac Disease & Irritable Bowel Syndrome 3.5 Diverticular Disease & Colorectal Cancer Screening 3.6 Viral Hepatitis, Steatotic & Alcohol-Associated Liver Disease 3.7 Cirrhosis & the Complications of Portal Hypertension 3.8 Acute Pancreatitis 3.9 Chronic Pancreatitis & Biliary Tract Disease 3.10 Inherited, Autoimmune & Drug-Induced Liver Disease 3.11 Small Intestinal Disease, Ischemia & Gastrointestinal Complications of HIV
5 Chapter 4: Infectious Disease (9% of Exam)
4.1 Lower Respiratory Tract Infections & Sepsis Syndromes 4.2 Viral Respiratory Pathogens & Tuberculosis 4.3 Central Nervous System Infections 4.4 Skin, Soft Tissue, Bone & Joint Infections 4.5 Infective Endocarditis & Cardiovascular Infection 4.6 Clostridioides difficile & Catheter-Related Bloodstream Infection 4.7 HIV Diagnosis, Antiretroviral Therapy & Prevention 4.8 Opportunistic Infections & Sexually Transmitted Infections 4.9 Fever of Unknown Origin, Neutropenic Fever & Toxic Shock 4.10 Travel-Related Illness, Vector-Borne Disease & Outbreaks 4.11 Infection Prevention, Nosocomial Infection & Postexposure Prophylaxis 4.12 Rheumatic Fever, Poststreptococcal Disease, Enteric & Hepatic Infections
6 Chapter 5: Pulmonary Disease (9% of Exam)
5.1 COPD: Diagnosis, GOLD Classification & Initial Pharmacotherapy 5.2 Interventions That Reduce COPD Mortality & Exacerbation Management 5.3 Asthma: Diagnosis, Stepwise Therapy & Status Asthmaticus 5.4 Interstitial Lung Disease & Idiopathic Pulmonary Fibrosis 5.5 Sarcoidosis, Hypersensitivity Pneumonitis & Occupational Lung Disease 5.6 Pulmonary Embolism: Diagnosis, Risk Stratification & Anticoagulation 5.7 Pulmonary Hypertension & Sleep-Disordered Breathing 5.8 Acute Respiratory Distress Syndrome & Mechanical Ventilation 5.9 Pleural Disease, Empyema & Pneumothorax 5.10 Bronchiectasis, Cystic Fibrosis & Restrictive Lung Disease 5.11 Evaluation of Cough, Dyspnea, Hemoptysis & the Solitary Pulmonary Nodule
7 Chapter 6: Rheumatology & Orthopedics (9% of Exam)
6.1 Rheumatoid Arthritis: Diagnosis & Systemic Manifestations 6.2 Antirheumatic Pharmacotherapy & the Spondyloarthropathies 6.3 Gout: Pathophysiology & Acute Management 6.4 Urate-Lowering Therapy & Calcium Pyrophosphate Deposition Disease 6.5 Osteoarthritis 6.6 Systemic Lupus Erythematosus 6.7 Systemic Sclerosis, Sjogren Syndrome & Inflammatory Myopathies 6.8 The Vasculitides 6.9 Regional Musculoskeletal Syndromes, Back Pain & Sports Injuries 6.10 Other Primary Rheumatic Disorders & Mixed Connective Tissue Disease 6.11 Osteonecrosis & Metabolic Bone Disease
8 Chapter 7: Hematology (6% of Exam)
7.1 Approach to Anemia & the Microcytic Anemias 7.2 Macrocytic, Normocytic & Hemolytic Anemias 7.3 Hemoglobinopathies, Thalassemias & Paroxysmal Nocturnal Hemoglobinuria 7.4 Platelet Disorders, Thrombocytopenia & the Bleeding Patient 7.5 Coagulation Factor Disorders, Thrombophilia & Anticoagulant Reversal 7.6 Acute Leukemias, Chronic Leukemias & Myeloproliferative Neoplasms 7.7 Lymphomas & Plasma Cell Disorders 7.8 Myelodysplastic Syndrome, Myeloproliferative Neoplasms & Porphyria 7.9 Transfusion Medicine & Late Complications of Transplantation
9 Chapter 8: Medical Oncology (6% of Exam)
8.1 Breast, Lung, Colorectal & Prostate Cancer 8.2 Oncologic Emergencies 8.3 Genitourinary & Gynecologic Malignancies 8.4 Head and Neck, Bone & Soft Tissue Cancer and Cancer of Unknown Primary 8.5 Complications of Cancer & Its Treatment 8.6 Cancer Prevention, Survivorship & Psychosocial Care
10 Chapter 9: Nephrology & Urology (6% of Exam)
9.1 Acute Kidney Injury: Staging, Etiology & Urine Microscopy 9.2 Glomerular Disorders & Nephrotic Syndrome 9.3 Nephritic Syndromes, RPGN & Indications for Renal Replacement Therapy 9.4 Chronic Kidney Disease: Staging & Progression Retardation 9.5 Complications of Chronic Kidney Disease: Anemia, CKD-MBD & Acidosis 9.6 Hyponatremia, Hypernatremia & Water Balance 9.7 Potassium Disorders & Systematic Acid-Base Analysis 9.8 Renal Tubular Acidosis & Nephrolithiasis 9.9 Prostate Disorders, Urinary Incontinence & Other Urologic Conditions 9.10 Cystic Kidney Disease, Obstructive Uropathy, Tubulointerstitial Disease & Hematuria
11 Chapter 10: Neurology (4% of Exam)
10.1 Acute Ischemic Stroke: Syndromes & Reperfusion 10.2 Transient Ischemic Attack, Secondary Prevention & Hemorrhagic Stroke 10.3 Headache & Facial Pain 10.4 Seizures, Epilepsy & Movement Disorders 10.5 Multiple Sclerosis & Neuromuscular Junction Disorders 10.6 Motor Neuron Disease & Acute Peripheral Neuropathy 10.7 Cranial Neuropathies & Neuro-Ophthalmology 10.8 Peripheral Neuropathies, CNS Tumors & Other Neurologic Disorders
12 Chapter 11: Psychiatry & Behavioral Health (4% of Exam)
11.1 Major Depressive Disorder & Medical Mimics 11.2 Antidepressant Pharmacotherapy & Treatment-Resistant Depression 11.3 Bipolar Disorder, Mood Stabilizers & Lithium Toxicity 11.4 Anxiety, Stress-Related & Obsessive-Compulsive Disorders 11.5 Alcohol Use Disorder & Withdrawal 11.6 Pharmacotherapy for Alcohol & Opioid Use Disorder 11.7 Toxidromes, Psychiatric Emergencies & Suicide Risk Assessment 11.8 Psychotic, Somatic Symptom, Factitious & Personality Disorders 11.9 Eating Disorders, Sleep Disorders & Sexual Health
13 Chapter 12: Dermatology (3% of Exam)
12.1 Dermatitis, Papulosquamous & Inflammatory Dermatoses 12.2 Acne, Rosacea & Cutaneous Infections 12.3 Herpes Zoster & Autoimmune Blistering Diseases 12.4 Nodules, Tumors & Cutaneous Oncology 12.5 Dermatologic Emergencies & Severe Cutaneous Adverse Reactions 12.6 Pigment Disorders, Alopecia, Nail Disease & Photosensitivity 12.7 Vascular Dermatoses, Environmental Injury & Cutaneous Signs of Systemic Disease
14 Chapter 13: Obstetrics & Gynecology / Women's Health (3% of Exam)
13.1 Contraception, Preconception Care & Teratogenicity 13.2 Hypertensive Disorders of Pregnancy & Gestational Diabetes 13.3 Thyroid Disease & Venous Thromboembolism in Pregnancy 13.4 Menopause & Menopausal Hormone Therapy 13.5 Abnormal Uterine Bleeding, Endometrial Disease & Gynecologic Cancer Screening 13.6 Breast Disorders & Diseases of the Vulva and Vagina 13.7 Menstrual Disorders, Fibroids, Endometriosis & Early Pregnancy Complications
15 Chapter 14: Geriatric Syndromes (3% of Exam)
14.1 Comprehensive Geriatric Assessment & Frailty 14.2 Falls, Gait Assessment & Polypharmacy 14.3 Urinary Incontinence & Pressure Injuries in Older Adults 14.4 Mild Cognitive Impairment, Dementia & Its Subtypes 14.5 Delirium & the Pharmacotherapy of Cognitive Disorders 14.6 Palliative Care, Hospice & Advance Care Planning 14.7 Chronic Wounds, Nutrition, Swallowing, Constipation & Hip Fracture 14.8 Sensory Impairment, Dizziness, Elder Mistreatment & Care Settings
16 Chapter 15: Allergy & Immunology (2% of Exam)
15.1 Anaphylaxis: Recognition & Emergency Management 15.2 Urticaria & Angioedema 15.3 Drug Allergy, Desensitization & Adult Immunodeficiency 15.4 Food Allergy, Insect Venom Hypersensitivity, Serum Sickness & Contrast Reactions 15.5 Rhinitis, Conjunctivitis, Eosinophilic & Mast Cell Disorders
17 Chapter 16: Preventive Medicine, Immunization & Clinical Epidemiology
16.1 The USPSTF Framework & Evidence-Based Cancer Screening 16.2 Cardiovascular & Metabolic Screening and Primary Prevention 16.3 Adult Immunization & Vaccines in Special Populations 16.4 Clinical Epidemiology, Biostatistics & Study Design
18 Chapter 17: Miscellaneous - Ethics, Patient Safety & Quality Improvement (2% of Exam)
17.1 Principles of Bioethics & Decisional Capacity 17.2 Informed Consent, Treatment Refusal & Special Scenarios 17.3 Surrogate Decision-Making, Advance Directives & Confidentiality 17.4 Patient Safety Science, Medical Error & Diagnostic Reasoning 17.5 Quality Improvement Methods, Health Disparities & Health Equity
19 Chapter 18: Perioperative Medicine & Medical Consultation
18.1 Preoperative Cardiovascular Risk Assessment 18.2 Preoperative Pulmonary Risk & Perioperative Medication Management 18.3 Perioperative Anticoagulation, Bridging & Postoperative Complications
20 Chapter 19: Ophthalmology (1% of Exam)
19.1 The Acute Red Eye & Ophthalmic Emergencies 19.2 Painless Vision Loss & Chronic Ophthalmic Disease 19.3 Uveitis, Scleritis & Orbital Disease
21 Chapter 20: Otolaryngology & Dental Medicine (1% of Exam)
20.1 Dizziness, Vertigo & Epistaxis 20.2 Ear Disorders, Hearing Loss & Upper Respiratory Infections 20.3 Oral Cavity, Salivary Gland, Laryngeal & Taste and Smell Disorders
1.11 Adult Congenital Heart Disease & Coarctation of the Aorta
Key Takeaways Explain This Topic AI
Atrial septal defect and patent foramen ovale, bicuspid aortic valve, patent ductus arteriosus, ventricular septal defect and pulmonic stenosis are the enumerated adult congenital blueprint topics.
A fixed, widely split second heart sound is the classic auscultatory signature of a secundum atrial septal defect.
Bicuspid aortic valve is associated with aortopathy, so the ascending aorta must be imaged even when valve function is normal.
Coarctation is listed under the hypertension subsection and presents with upper-extremity hypertension, radiofemoral delay and rib notching.
Once shunt reversal produces Eisenmenger physiology, defect closure is contraindicated because the shunt has become the right ventricle's pressure-relief pathway.
Last updated: August 2026
1. Why Adult Congenital Disease Appears on an Internal Medicine Exam
More adults than children now live with congenital heart disease, and most are cared for by internists between specialist visits. ABIM lists Congenital heart disease in adults as a named subsection under Cardiovascular Disease, enumerating atrial septal defect and patent foramen ovale, bicuspid aortic valve , and other congenital heart disease (patent ductus arteriosus, ventricular septal defect, pulmonic stenosis) . Coarctation of the aorta is listed separately, under the hypertension subsection.
These items are usually recognition questions built on a physical finding, an ECG pattern or an incidental imaging result — not management questions requiring surgical judgment.
2. Atrial Septal Defect and Patent Foramen Ovale
A patent foramen ovale (PFO) is a normal fetal structure that fails to seal; it is present in roughly a quarter of adults and is not a defect in the anatomic sense. An atrial septal defect (ASD) is a true deficiency of the septum. The distinction matters because it drives both physiology and management.
- Feature Secundum ASD PFO
- Anatomy True septal tissue deficiency Unsealed flap valve
- Shunt Continuous left-to-right Intermittent right-to-left with strain
- Chamber effect Right atrial and ventricular dilation None
- Classic exam finding Fixed, widely split S2 None
Typical clinical issue Exercise intolerance, atrial arrhythmia, late pulmonary hypertension Cryptogenic stroke, decompression illness
The fixed split S2 is the single highest-yield finding. In normal physiology, inspiration delays pulmonic valve closure and widens the split; expiration narrows it. A large left-to-right atrial shunt keeps right ventricular volume loaded throughout the respiratory cycle, so the split no longer varies. A systolic murmur may be present, but it arises from increased flow across the pulmonic valve, not from flow across the defect itself — the interatrial pressure gradient is too small to generate a murmur.
The ECG helps localize the defect: secundum defects typically show right axis deviation with an incomplete right bundle branch pattern, whereas primum defects show left axis deviation and are associated with atrioventricular valve clefts and with Down syndrome.
Closure of an ASD is considered when there is right-sided chamber enlargement, generally with a shunt fraction (Qp:Qs) above 1.5:1, provided pulmonary vascular resistance has not risen prohibitively. PFO closure is a different question entirely and is considered chiefly in younger patients after cryptogenic stroke when no other mechanism is found.
3. Bicuspid Aortic Valve
A bicuspid aortic valve is the most common congenital cardiac malformation. Two exam points dominate:
It stenoses early. Turbulent flow across two rather than three cusps accelerates calcific degeneration, so bicuspid patients present with severe aortic stenosis one to two decades earlier than patients with trileaflet valves. An ejection click followed by a systolic murmur in a patient in their fifties should raise the possibility.
The aortopathy is independent of the valve lesion. Bicuspid valve disease is associated with an intrinsic abnormality of the aortic media, so ascending aortic dilation, aneurysm and dissection occur even when the valve works normally . The ascending aorta must therefore be imaged and followed regardless of valve function, and first-degree relatives should be screened.
4. Ventricular Septal Defect, Patent Ductus Arteriosus and Pulmonic Stenosis
Lesion Characteristic murmur Key associations
Ventricular septal defect Harsh holosystolic murmur, left lower sternal border Smaller defects are louder; endocarditis risk
Patent ductus arteriosus Continuous "machinery" murmur, left infraclavicular Differential cyanosis if shunt reverses
Pulmonic stenosis Systolic ejection murmur, left upper sternal border, increases with inspiration Ejection click that softens with inspiration
A counterintuitive rule worth memorizing: in ventricular septal defect, murmur intensity is inversely related to defect size . A small restrictive defect generates a high-velocity jet and a loud murmur; a large defect equalizes ventricular pressures and may be nearly silent while causing far more physiologic harm.
Right-sided murmurs — pulmonic stenosis, tricuspid regurgitation — increase with inspiration as venous return to the right heart rises. Left-sided murmurs do not. This is the fastest bedside discriminator on the exam.
5. Coarctation of the Aorta
Coarctation is listed by ABIM under Hypertension , and that placement is the clinical clue: it is a surgically correctable cause of secondary hypertension in a young patient.
The triad to recognize:
Upper-extremity hypertension with lower-extremity hypotension. A systolic gradient between arm and leg is the defining finding.
Radiofemoral delay. The femoral pulse is delayed and diminished relative to the radial pulse.
Rib notching on chest radiograph. Dilated intercostal collateral arteries erode the inferior rib margins, and the dilated aorta above and below the narrowing can produce a "figure 3" contour.
Coarctation is strongly associated with bicuspid aortic valve (present in a majority of cases) and with intracranial berry aneurysms; it is also a recognized feature of Turner syndrome. Any young hypertensive patient should have blood pressure measured in an arm and a leg at least once — it is a two-minute test that occasionally reveals a curable cause.
6. Eisenmenger Physiology
A long-standing, uncorrected left-to-right shunt drives pulmonary vascular remodeling. When pulmonary vascular resistance rises above systemic resistance, the shunt reverses and the patient becomes cyanotic — Eisenmenger syndrome .
The critical management consequence: once Eisenmenger physiology is established, defect closure is contraindicated. The shunt has become the right ventricle's only pressure-relief pathway; closing it converts a cyanotic but compensated circulation into acute right ventricular failure.
Other management principles in Eisenmenger physiology:
Avoid systemic vasodilation and volume depletion, both of which increase right-to-left shunting and worsen hypoxemia.
Avoid routine phlebotomy for erythrocytosis; the elevated hematocrit is a compensatory response to hypoxemia, and phlebotomy causes iron deficiency and increases stroke risk.
Use meticulous air-filter precautions on intravenous lines, because a right-to-left shunt allows paradoxical air embolism.
Pregnancy carries very high maternal mortality and is generally advised against.
Differential cyanosis — pink fingers with blue toes — is the signature of a reversed patent ductus arteriosus, because the deoxygenated shunt enters the aorta distal to the vessels supplying the upper extremities.