Curriculum Heart Diseases

For Paola

Hypertrophic

 

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Dressler’s Syndrome Post-Injury Syndrome Post-MI Syndrome
Post-Pericardiotomy Syndrome
Definition: Delayed autoimmune pleuropericarditis occurring weeks to months after myocardial infarction or cardiac surgery.
Comment: Body develops antibodies against pericardial antigens exposed during injury.
Effusion: Pericardial and pleural effusions
Enhancement: Thickened, enhancing pericardium
Lung: Basal consolidation
Pericardium Inflam Dressler’s Syndrome
Libman-Sacks Endocarditis Autoimmune Valvulitis SLE Endocarditis
Verrucous Endocarditis
Definition: Sterile, inflammatory vegetations associated with Systemic Lupus Erythematosus (SLE).
Comment: Can occur on the undersurface of valves (unlike infective endocarditis).
Vegetation: Small, wart-like masses
Position: Both sides of valve leaflets (atrial and ventricular)
Distribution: Mitral valve most common
Mitral / Aortic Valve Inflam Mitral Valve Pathology
Myocarditis (Giant Cell/Autoimmune) Inflammatory Myopathy Fulminant Myocarditis Definition: Severe, often fatal autoimmune inflammation of the myocardium characterized by multinucleated giant cells.
Comment: Often presents with rapidly progressive heart failure or arrhythmia.
Function: Rapidly progressive heart failure (dilated/hypokinetic)
MRI: Diffuse Late Gadolinium Enhancement (LGE)
Edema: High T2 signal
Myocardium Inflam Myocarditis
Pericarditis (Acute) Pericardial Inflammation Viral Pericarditis
Idiopathic Pericarditis
Definition: Acute inflammation of the pericardial layers, most commonly viral or idiopathic in origin.
Comment: Presents with pleuritic chest pain relieved by leaning forward.
Enhancement: Thickened, avidly enhancing pericardium
Fluid: Small to moderate simple effusion
Fat: Stranding of epicardial fat
Pericardium Inflam Acute Pericarditis
Rheumatic Heart Disease Post-Streptococcal Rheumatic Fever Definition: Chronic valve damage resulting from an autoimmune reaction to Group A Beta-hemolytic Streptococcus.
Comment: The Mitral valve is affected in nearly all cases; Aortic in 20-30%.
MV: Commissural fusion (“Hockey Stick”), Stenosis
AV: Leaflet thickening, triangular systolic orifice
LA: Enlargement and calcification
Valves (Mitral/Aortic) Inflam Rheumatic Heart Disease

Case 014 (87M)

Case 014H
Sarcoidosis (Cardiac) Granulomatous Cardiac Sarcoid Definition: Infiltration of the myocardium by non-caseating granulomas.
Comment: Can mimic MI but typically does not follow coronary territories. “The Great Imitator.”
MRI: Patchy, mid-wall or epicardial LGE (non-coronary)
PET: Focal FDG uptake (active phase)
Nodes: Hilar lymphadenopathy
Myocardium / Conduction Inflam Sarcoidosis Overview

Case 016 (57M Sarcoid)

Case 016H

 

Inflammation and Immune Disorders of the Heart

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Chagas Disease Parasitic Infection American Trypanosomiasis Definition: Chronic cardiomyopathy caused by the parasite Trypanosoma cruzi.
Comment: Endemic to Latin America. A classic cause of apical aneurysms in the absence of CAD.
Structure: Apical Aneurysm (Left Ventricle)
Function: Dilated Cardiomyopathy
GI: Associated megaesophagus/megacolon
Left Ventricle Infection LV Aneurysm (Causes)
Hydatid Cyst (Cardiac) Parasitic Infection Echinococcosis Definition: Cystic infestation of the myocardium by Echinococcus granulosus.
Comment: Rupture can cause anaphylactic shock.
Character: Multiloculated cystic mass (“Mother and Daughter” cysts)
Wall: Calcified rim
Position: Intra-myocardial (IVS or LV wall)
Myocardium Infection Cardiac Cysts
Infective Endocarditis Bacterial Infection Bacterial Endocarditis
SBE
Definition: Microbial infection of the endocardial surface, typically the valves, forming vegetations.
Comment: Staph aureus (acute) and Strep viridans (subacute) are common pathogens.
Mass: Mobile, oscillating vegetation
Destruction: Valve perforation or paravalvular abscess
Emboli: Septic emboli to lungs (Right) or brain/kidney (Left)
Valves Infection Bacterial Endocarditis
Myocarditis (Viral) Viral Infection Coxsackie Myocarditis Definition: Direct viral injury and subsequent immune response damaging the myocardium.
Comment: MRI is the gold standard for non-invasive diagnosis (Lake Louise Criteria).
MRI: T2 high signal (Edema) + Early/Late Gadolinium Enhancement
Pattern: Epicardial or mid-wall (Lake Louise Criteria)
Function: Global hypokinesis
Myocardium Infection Viral Myocarditis
Pericarditis (Tuberculous) Mycobacterial Infection TB Pericarditis Definition: Chronic infection of the pericardium by Mycobacterium tuberculosis.
Comment: A common cause of constrictive pericarditis in developing nations.
Character: Grossly thickened, irregular pericardium
Calcification: Extensive (“Armor Heart”)
Function: Constrictive physiology (ventricular interdependence)
Pericardium Infection Constrictive Pericarditis
Syphilitic Aortitis Spirochete Infection Luetic Aortitis Definition: Tertiary syphilis affecting the vasa vasorum of the ascending aorta.
Comment: Leads to destruction of the media and “tree-barking” of the intima.
Calcification: “Tree-bark” linear calcification of Ascending Aorta
Shape: Saccular aneurysm
Valve: Aortic regurgitation
Aorta (Ascending) Infection Aortic Aneurysms

 

Infections of the Heart

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Linkable Page (Context) Linkable Case Examples
Cardiac Tamponade Compression Pericardial Tamponade Definition: Hemodynamic compromise caused by fluid accumulation in the pericardial space compressing the heart.
Comment: The rate of accumulation is more critical than the volume.
Chambers: Diastolic collapse of RA and RV (early diastole)
IVC: Dilated with no respiratory variation (plethora)
Septum: Septal bounce (ventricular interdependence)
Pericardium Mechanical Tamponade Case 88H
Prosthetic Dehiscence Device Failure Valve Dehiscence
Paravalvular Leak
Definition: Separation of the sewing ring of a prosthetic valve from the tissue annulus.
Comment: Often caused by infection (ring abscess) or suture failure.
Motion: “Rocking” motion of the valve on cine imaging
Flow: Paravalvular regurgitation (leak outside the ring)
CT: Perivalvular hypoattenuation (abscess)
Prosthetic Valve Mechanical Prosthetic Valves
Tension Pneumothorax Extrinsic Compression Tension PTX Definition: Accumulation of air under pressure in the pleural space, compressing the mediastinum.
Comment: A life-threatening emergency requiring immediate decompression.
Shift: Mediastinal shift away from the air
Shape: Depression/Inversion of the hemidiaphragm
Heart: Compression of the RA/RV impairing filling
Pleura / Mediastinum Mechanical Faces of Pneumothorax Case 92L

 

Maligbnat and Benign Tumors of the Heart

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Angiosarcoma Malignant Neoplasm Primary Cardiac Sarcoma Definition: The most common primary malignant tumor of the heart, arising from endothelial cells.
Comment: Highly aggressive with a predilection for the Right Atrium.
Character: Large, heterogeneous, invasive mass
Enhancement: “Sunray” appearance (avid/heterogeneous)
Effusion: Hemorrhagic pericardial effusion
Right Atrium Neoplasm (Malignant) Faces of Angiosarcoma
Lymphoma (Primary) Malignant Neoplasm Cardiac Lymphoma Definition: Extranodal Non-Hodgkin Lymphoma involving the heart.
Comment: Often associated with immunocompromised states (HIV/Post-transplant).
Character: Homogeneous, infiltrating soft tissue mass
Location: Right Atrium / AV Groove
Effect: Encircles vessels/coronaries without compressing them (“Sandwich sign”)
Right Atrium Neoplasm (Malignant) Faces of Lymphoma
Metastasis (Cardiac) Malignant Neoplasm Secondary Tumor Definition: Spread of cancer from a distant primary site to the heart or pericardium.
Comment: 20-40x more common than primary cardiac tumors. Common sources: Lung, Breast, Melanoma.
Location: Pericardium (Effusion/Nodules) > Myocardium
Appearance: Multiple nodules or diffuse infiltration
Enhancement: Enhances with contrast (unlike thrombus)
Pericardium / Myocardium Neoplasm (Malignant) Faces of Metastasis
Fibroma Benign Neoplasm Cardiac Fibroma Definition: A benign, solitary congenital tumor composed of fibroblasts and collagen.
Comment: Second most common pediatric tumor. Can cause arrhythmias or obstruction.
Character: Homogeneous, hypointense (dark) on T2 MRI
Calcification: Central calcification is common
Location: Ventricular septum or free wall
Ventricles Neoplasm (Benign) Faces of Fibroma
Lipoma Benign Neoplasm Fatty Tumor Definition: Encapsulated benign tumor composed of mature adipocytes.
Comment: Distinct from Lipomatous Hypertrophy (which is non-encapsulated septal fat).
Density: Homogeneous fat density (-100 HU)
Signal: High T1/T2 (suppresses with Fat Sat)
Location: Subendocardial, intramuscular, or epicardial
Atrium / Ventricle Neoplasm (Benign) Faces of Lipoma
Myxoma Benign Neoplasm Atrial Myxoma Definition: The most common primary cardiac tumor (benign, gelatinous).
Comment: 75% occur in Left Atrium attached to Fossa Ovalis. Can embolize or obstruct the mitral valve (mimicking MS).
Attachment: Pedunculated stalk on IAS
Motion: Mobile; may prolapse through valve
Character: Heterogeneous enhancement; calcification rare
Left Atrium Neoplasm (Benign) Faces of Myxoma Case 044H
Case 045H
Papillary Fibroelastoma Benign Neoplasm Cardiac Papilloma Definition: Small, benign valvular tumor resembling a sea anemone.
Comment: The most common tumor of the heart valves. High risk of embolization (stroke/MI) despite being benign.
Location: Aortic or Mitral Valve leaflets
Size: Small (< 1 cm), mobile, pedunculated
Motion: “Shimmy” or vibration on Echo
Valves Neoplasm (Benign) Faces of Fibroelastoma
Rhabdomyoma Benign Neoplasm Hamartoma Definition: Benign hamartoma of cardiac myocytes.
Comment: The most common pediatric heart tumor. Strongly associated with Tuberous Sclerosis. Often regress spontaneously.
Number: Often multiple
Location: Ventricular walls or Septum
Echo: Hyperechoic (bright) nodules
Ventricles Neoplasm (Benign) Faces of Rhabdomyoma

 

 

Mechanical Abnormalities

Finding Definition & Comment (P-SSPCT-C) Diagnosis Mod Cat Linkable Page (Context) Linkable Image (Game)
Compression, Tamponade Definition: Accumulation of pericardial fluid under pressure, compressing cardiac chambers.
Comment: Diastolic collapse of the RA (early) and RV (late) is diagnostic. The IVC is dilated (plethora) without respiratory variation.
Cardiac Tamponade Echo/CT Compression Tamponade Case 88H
Compression, Tension PTX Definition: Accumulation of air under pressure in the pleural space.
Comment: Causes mediastinal shift away from the air, compressing the SVC/IVC and impairing venous return (Obstructive Shock).
Tension Pneumothorax CXR/CT Compression Tension Pneumothorax Case 92L
Restriction, Constrictive Definition: Thickened, calcified pericardium limiting diastolic filling.
Comment: Causes Ventricular Interdependence (septal bounce) where the RV and LV compete for fixed space. “Square root sign” on pressure tracing.
Constrictive Pericarditis CT/MRI Restriction Constrictive Pericarditis
Rupture, Papillary Muscle Definition: Tear of the papillary muscle body or head, usually 3-7 days post-MI.
Comment: Causes acute, severe Mitral Regurgitation. The Posteromedial muscle (supplied only by RCA) ruptures more often than the Anterolateral (dual supply).
Papillary Muscle Rupture Echo Rupture Acute Mitral Regurgitation Case 57H
Rupture, Pseudoaneurysm Definition: Contained rupture of the ventricular free wall.
Comment: The wall is composed of pericardium and thrombus (no myocardium). Communicates with the ventricle via a narrow neck. High risk of rupture.
Ventricular Pseudoaneurysm CT/MRI Rupture RV Pseudoaneurysm Case Case 30F
Rupture, Septal (VSR) Definition: Tear in the Interventricular Septum following transmural MI.
Comment: Creates an acute Left-to-Right shunt. Clinically presents with a new harsh holosystolic murmur and hypotension.
Ventricular Septal Rupture Echo/CT Rupture Ventricular Septal Defects
Failure, Dehiscence Definition: Separation of a prosthetic valve sewing ring from the tissue annulus.
Comment: Causes “Rocking” motion of the valve and paravalvular leak. Often due to infective endocarditis (ring abscess).
Prosthetic Valve Dehiscence Echo/CT Device Prosthetic Valves

 

Trauma to the Heart

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Aortic Transection Vascular Injury Traumatic Aortic Rupture Definition: Near-complete tear of the aortic wall layers caused by rapid deceleration.
Comment: Most commonly occurs at the aortic isthmus due to tethering by the Ligamentum Arteriosum. High mortality at scene.
Direct: Pseudoaneurysm, intimal flap, or contour irregularity at isthmus
Indirect: Mediastinal hematoma, apical capping
Aorta (Isthmus) Trauma Traumatic Aortic Injury
Cardiac Contusion Myocardial Injury Myocardial Bruise Definition: Bruising of the myocardium following blunt chest trauma (e.g., steering wheel impact).
Comment: The Right Ventricle is most commonly injured due to its anterior position behind the sternum. Can mimic MI clinically.
MRI: Focal edema (High T2) and LGE matching impact site
Echo: Focal wall motion abnormality (often RV)
Myocardium (RV) Trauma Cardiac Trauma
Foreign Body (Penetrating) Penetrating Injury Bullet / Shrapnel / Needle Definition: Presence of an external object within the cardiac structures.
Comment: Can be acute (GSW) or chronic (IV drug user with needle embolus). Risk of erosion, tamponade, or infection.
CT: High-density streak artifact from metallic object
Location: Intracavitary, intramural, or pericardial
Heart (Any) Trauma Foreign Body (Needle Case) Case 48M
Hemopericardium Pericardial Injury Blood in Pericardium Definition: Accumulation of blood within the pericardial sac.
Comment: In trauma, usually indicates cardiac rupture or coronary artery laceration. Rapid accumulation leads to tamponade.
CT: High-density fluid (60-80 HU) in pericardial space
US: Echogenic fluid with clot strands
Pericardium Trauma Hemopericardium
Pneumopericardium Pericardial Injury Air in Pericardium Definition: Presence of air within the pericardial sac.
Comment: Indicates communication with the airway (tracheobronchial rupture), esophagus, or penetrating chest wall injury.
CXR: “Halo sign” of continuous air band surrounding the heart
CT: Air density anterior to the RV
Pericardium Trauma Pneumopericardium Case 31M
Traumatic VSD Myocardial Injury Septal Rupture Definition: Tear in the interventricular septum caused by severe blunt compression or penetrating trauma.
Comment: Results in an acute Left-to-Right shunt and potential heart failure.
Doppler: High-velocity systolic jet across septum
CT: Contrast jet extending from LV to RV
Septum Trauma Traumatic VSD

 

Metabolic Diseases of the Heart

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Amyloidosis (Cardiac) Infiltrative / Storage Stiff Heart Syndrome
ATTR / AL Amyloid
Definition: Extracellular deposition of insoluble amyloid fibrils within the myocardial interstitium.
Comment: Causes a restrictive cardiomyopathy. The “Sparkling” texture on Echo is classic but nonspecific.
MRI: Diffuse subendocardial or transmural LGE; inability to null myocardium (T1)
Size: Concentric bi-ventricular thickening
Atria: Bi-atrial enlargement and septal thickening
Myocardium Metabolic Faces of Amyloidosis Case 70F
(Amyloid CT/MRI)
Carcinoid Heart Disease Hormonal / Toxic Hedinger Syndrome Definition: Fibrous plaque deposition on heart valves caused by high circulating serotonin from neuroendocrine tumors.
Comment: Sparing of the left heart is typical (unless a PFO exists) because lungs inactivate serotonin.
Valves: Thickened, retracted, fixed leaflets (Tricuspid/Pulmonic)
Function: Mixed severe Regurgitation and Stenosis
Liver: Metastases usually present
Tricuspid / Pulmonic Metabolic Faces of Carcinoid Case 118 (56M)
(Tip of Iceberg)
Fabry Disease Storage Disease Anderson-Fabry Definition: X-linked lysosomal storage disorder (deficiency of alpha-galactosidase A) causing glycosphingolipid accumulation.
Comment: Mimics Hypertrophic Cardiomyopathy (HCM). Low T1 mapping values are pathognomonic.
MRI (LGE): Mid-wall fibrosis in the basal inferolateral wall (classic)
Character: Low native T1 values (unlike other hypertrophies which are high)
Size: Concentric LV Hypertrophy
Left Ventricle Metabolic Concentric HCM (Fabry) Case 64F
(Heterozygote)
Hemochromatosis Storage Disease Iron Overload Cardiomyopathy Definition: Intracellular accumulation of iron within cardiomyocytes leading to toxicity and fibrosis.
Comment: Reversible cause of heart failure. “Bronze Diabetes” is the clinical triad.
MRI (T2*): Myocardium appears black (hypointense) due to iron effect
Function: Restrictive (early) to Dilated (late) pattern
Liver: Concomitant iron overload (dark liver)
Myocardium Metabolic Dilated Cardiomyopathy Case 41M
(Iron Overload)
Glycogen Storage Disease Storage Disease Pompe Disease (Type II) Definition: Inherited deficiency of acid alpha-glucosidase leading to massive glycogen accumulation in lysosomes.
Comment: Presents in infancy with massive cardiomegaly and hypotonia (“floppy baby”).
Size: Massive, generalized biventricular hypertrophy
Function: Severe obstruction and eventual systolic failure
EKG: Giant QRS complexes and short PR interval
Ventricles Metabolic Pediatric Cardiomyopathy

 

 

Circulatory Diseases of the Heart

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Acute Myocardial Infarction Ischemic Heart Disease Heart Attack
STEMI / NSTEMI
Definition: Myocardial necrosis due to acute obstruction of a coronary artery.
Comment: “Time is Muscle.” Complications include rupture, VSD, and aneurysm.
MRI: Subendocardial or Transmural LGE (vascular territory)
Echo: Regional Wall Motion Abnormality (RWMA)
Edema: High T2 signal (acute)
Myocardium Circulatory Myocardial Infarction Case 004H
Coronary Artery Anomaly (High Risk) Congenital / Vascular Anomalous Origin Definition: Origin of a coronary artery from the opposite cusp causing an inter-arterial course.
Comment: The vessel courses *between* the Aorta and PA. High risk of sudden death in young athletes.
CT: “Inter-arterial” course
Shape: Slit-like orifice or acute take-off angle
Structure: RCA from Left Cusp (or LCA from Right)
Coronary Arteries Circulatory Coronary Anomalies
Ischemic Cardiomyopathy Ischemic Heart Disease Post-MI Heart Failure Definition: Global ventricular dysfunction resulting from severe CAD or prior infarction.
Comment: The most common cause of heart failure. Distinct from Dilated CM by the presence of scar.
MRI: Subendocardial scarring (LGE) following vascular territories
Shape: Remodeling / Dilation
Function: Reduced EF
Left Ventricle Circulatory Ischemic Cardiomyopathy
Cardiogenic Shock Hemodynamic Failure Pump Failure Definition: State of end-organ hypoperfusion due to cardiac pump failure.
Comment: Commonly caused by massive MI, acute MR, or tamponade. Mortality is high.
Lungs: Pulmonary Edema (Batwing)
Heart: Dilated, poorly contractile chambers (or mechanical defect)
IVC: Dilated (Plethora)
Systemic Shock Heart Failure & Shock
ARVC Cardiomyopathy Arrhythmogenic RV Dysplasia Definition: Genetic replacement of RV myocardium with fibro-fatty tissue.
Comment: “Triangle of Dysplasia” (Inflow, Outflow, Apex). Major cause of sudden death in young people.
MRI: Fatty infiltration / Wall thinning
Shape: Aneurysmal outpouchings (“scalloping”)
Function: RV dyskinesis
Right Ventricle Cardiomyopathy ARVC
Dilated Cardiomyopathy Cardiomyopathy DCM
Congestive CM
Definition: Ventricular dilation and impaired contraction (systolic failure) not explained by CAD or loading conditions.
Comment: Causes: Idiopathic, Viral, Alcohol, Peripartum.
Size: Globular enlargement (LVIDd > 5.8cm)
MRI: Mid-wall fibrosis (LGE) or no scar
Valve: Functional MR/TR
Ventricles (LV/RV) Cardiomyopathy Dilated CM Case 007
Hypertrophic Cardiomyopathy (HCM) Cardiomyopathy HOCM / IHSS Definition: Genetic disorder defined by inappropriate LV hypertrophy (>15mm) without a cause.
Comment: Often Asymmetric Septal Hypertrophy (ASH). Risk of LVOT obstruction (SAM).
Shape: Septal thickening > 1.5x posterior wall
MRI: Patchy fibrosis at insertion points
Motion: Systolic Anterior Motion (SAM) of MV
Left Ventricle Cardiomyopathy Hypertrophic CM Case 068H
(Apical HCM)
Restrictive Cardiomyopathy Cardiomyopathy Stiff Heart Definition: Primary diastolic dysfunction with preserved systolic function and normal wall thickness.
Comment: Least common CM. Causes: Amyloid, Sarcoid, Radiation, Endomyocardial Fibrosis.
Atria: Bi-atrial enlargement (massive)
Ventricles: Normal size, thick walls
Function: Diastolic Failure
Myocardium Cardiomyopathy Restrictive CM
Takotsubo Cardiomyopathy Cardiomyopathy Broken Heart Syndrome
Stress Cardiomyopathy
Definition: Transient regional systolic dysfunction mimicking MI, triggered by emotional stress.
Comment: Classic “Apical Ballooning” with basal hyperkinesis. Coronaries are normal.
Shape: “Octopus Pot” (narrow neck, ballooned apex)
Motion: Apical akinesis
Time: Recovers in weeks
Left Ventricle Cardiomyopathy Takotsubo Case 021H

 

 

Congenital

Congenital: SVC, IVC, Right Atrium, Tricuspid Valve

Disease / Diagnosis Definition & Comment (P-SSPCT-C) Grouped Category Mod Cat Linkable Page (Context) Linkable Image (Game)
Interrupted IVC (Azygos Cont.) Definition: Absence of the hepatic segment of the IVC with drainage via a dilated Azygos vein.
Connections: Hepatic veins drain directly into RA; Lower body drains via Azygos to SVC.
Comment: Often associated with Polysplenia / Heterotaxy syndromes.
Venous Anomaly CT/MRI Congenital IVC Anomalies
Persistent Left SVC Position: Vertical vessel lateral to the Aortic Arch, draining into Coronary Sinus.
Connections: Drains Left Jugular/Subclavian -> Coronary Sinus -> RA.
Comment: Most common thoracic venous anomaly. Causes a dilated Coronary Sinus.
Venous Anomaly CT/CXR Congenital Persistent Left SVC
Scimitar Syndrome (PAPVR) Shape: Curvilinear vessel (“Turkish Sword”) along right heart border.
Connections: Right Pulmonary Veins drain into IVC.
Comment: Associated with Right Lung Hypoplasia (Hypogenetic Lung Syndrome).
Venous Anomaly CXR/CT Congenital Scimitar Syndrome
ASD, Primum Position: Defect in the lower atrial septum (near AV valves).
Time: Failure of septum primum to fuse with endocardial cushions.
Comment: Part of the AV Septal Defect (Endocardial Cushion Defect) spectrum.
Atrial Septal Defect Echo Congenital ASD Types
ASD, Secundum Position: Defect at the Fossa Ovalis (central septum).
Definition: Most common ASD (70%).
Comment: Causes Left-to-Right shunt and RV volume overload (RVE).
Atrial Septal Defect Echo/MRI Congenital Secundum ASD
ASD, Sinus Venosus Position: High in the septum near SVC junction (Superior) or IVC (Inferior).
Connections: Almost always associated with PAPVR (RUPV into SVC).
Comment: Not a true septal defect, but a deficiency of the wall between SVC and LA.
Atrial Septal Defect CT/MRI Congenital Sinus Venosus Defect
Cor Triatriatum Dexter Parts: Membrane dividing the Right Atrium.
Time: Persistence of the right valve of the sinus venosus.
Comment: Rare. Can obstruct the Tricuspid Valve or IVC.
RA Anomaly Echo Congenital Cor Triatriatum
Ebstein’s Anomaly Position: Apical displacement of the septal tricuspid leaflet.
Parts: “Atrialized” portion of RV reduces functional RV volume.
Shape: “Box-shaped” heart on CXR.
Comment: Associated with WPW and PFO/ASD.
TV Anomaly CXR/MRI Congenital Ebstein’s Anomaly
Tricuspid Atresia Definition: Complete absence of the Tricuspid Valve (no RA-RV connection).
Connections: Requires ASD (for RA output) and VSD/PDA (for pulmonary blood flow).
Comment: Causes Hypoplastic Right Ventricle.
TV Anomaly Echo Congenital Tricuspid Atresia

Congenital: RV, RVOT, Conotruncal

Disease / Diagnosis Definition & Comment (P-SSPCT-C) Grouped Category Mod Cat Linkable Page (Context) Linkable Image (Game)
Double Outlet RV (DORV) Connections: Both Aorta and Pulmonary Artery arise predominantly (>50%) from the RV.
Definition: A spectrum of defects usually involving a VSD.
Comment: Physiology depends on VSD location (Subaortic vs Subpulmonic).
Conotruncal MRI/Echo Congenital DORV
Pulmonary Atresia (w/ VSD) Definition: Complete obstruction of RVOT with a VSD.
Comment: Considered severe Tetralogy of Fallot. Pulmonary blood flow supplied by MAPCAs (collaterals) or PDA.
Conotruncal Angio/CT Congenital Pulmonary Atresia
Tetralogy of Fallot (TOF) Parts: 1. VSD, 2. Overriding Aorta, 3. RVOT Obstruction (PS), 4. RV Hypertrophy.
Shape: “Boot Shaped Heart” (Coeur en sabot) due to uplifted apex.
Comment: Most common cyanotic heart disease.
Conotruncal CXR/CT Congenital Tetralogy of Fallot
Transposition (D-TGA) Connections: Ventriculo-Arterial Discordance (Aorta from RV, PA from LV).
Shape: “Egg on a String” appearance on CXR (narrow mediastinum).
Comment: Parallel circuits; incompatible with life without mixing (ASD/VSD/PDA).
Conotruncal CXR/Echo Congenital Transposition (TGA)
Truncus Arteriosus Parts: Single arterial trunk arising from the heart supplying Coronaries, Aorta, and PAs.
Time: Failed septation of the embryonic truncus.
Comment: Always associated with a large VSD.
Conotruncal CT/MRI Congenital Truncus Arteriosus
Pulmonary Stenosis (Valvular) Shape: Systolic doming of leaflets.
Connections: Post-stenotic dilation of MPA/LPA.
Comment: Can be isolated or part of syndromes (Noonan’s).
RVOT Obstruction Echo Congenital Pulmonary Stenosis
RV Hypoplasia Size: Small, under-developed RV cavity.
Connections: Secondary to Tricuspid Atresia or Pulmonary Atresia with intact septum.
Comment: Requires univentricular palliation (Fontan).
Ventricular Defect MRI Congenital Hypoplastic Right Heart

Congenital: LA, MV, Left Ventricle

Disease / Diagnosis Definition & Comment (P-SSPCT-C) Grouped Category Mod Cat Linkable Page (Context) Linkable Image (Game)
Cor Triatriatum Sinister Parts: Membrane dividing LA into proximal (veins) and distal (appendage/valve) chambers.
Comment: Presents like Mitral Stenosis. Rare.
LA Anomaly Echo Congenital Cor Triatriatum
Cleft Mitral Valve Shape: Cleft/split in the Anterior Mitral Leaflet.
Connections: Causes Mitral Regurgitation.
Comment: Classic feature of Partial AV Septal Defect (Primum ASD).
MV Anomaly Echo Congenital Cleft Mitral Valve
Parachute Mitral Valve Connections: All chordae attach to a single papillary muscle.
Shape: Funnel-like stenosis.
Comment: Component of Shone’s Complex (Multiple left-sided obstructions).
MV Anomaly Echo Congenital Congenital MV Anomalies
Hypoplastic Left Heart (HLHS) Size: Severe underdevelopment of LV, Mitral Valve, Aortic Valve, and Aortic Arch.
Connections: Systemic flow dependent on PDA.
Comment: Critical ductal-dependent lesion.
Ventricular Defect Echo/MRI Congenital HLHS
LV Non-Compaction Character: Deep trabecular recesses (“Spongy” myocardium).
Size: Non-compacted to Compacted ratio > 2.3.
Time: Arrest of compaction (Week 5-8).
Cardiomyopathy MRI Congenital Non-Compaction
VSD (Membranous) Position: Upper septum (in membranous portion).
Definition: Most common VSD type (70%).
Comment: Often closes spontaneously. Small defects cause loud murmurs (Maladie de Roger).
Ventricular Septal Defect Echo Congenital VSD Types

Congenital: LVOT, Aorta, Coronaries

Disease / Diagnosis Definition & Comment (P-SSPCT-C) Grouped Category Mod Cat Linkable Page (Context) Linkable Image (Game)
Bicuspid Aortic Valve Parts: 2 Cusps (Fusion of R-L most common).
Shape: “Football” orifice; Systolic doming.
Comment: Associated with Aortic Coarctation and Ascending Aortic Aneurysm.
Valvular Anomaly Echo/MRI Congenital Bicuspid Valve
Subaortic Membrane Parts: Fibrous ridge or membrane below the AV.
Connections: High velocity jet causing damage to AV leaflets (AR).
Comment: Discrete Subaortic Stenosis.
LVOT Obstruction Echo Congenital Subaortic Stenosis
Coarctation of Aorta Size: Focal narrowing of aortic isthmus.
Shape: “3 Sign” on CXR.
Connections: Rib notching (intercostal collaterals).
Comment: Causes upper limb HTN and lower limb hypotension.
Aortic Arch Anomaly CT/CXR Congenital Coarctation
Double Aortic Arch Parts: Aorta splits into R and L arches encircling trachea/esophagus.
Shape: Complete vascular ring.
Comment: Causes compression symptoms (stridor, dysphagia).
Vascular Ring CT/MRI Congenital Vascular Rings
Patent Ductus Arteriosus Connections: Communication between Descending Aorta and Left PA.
Time: Failure of fetal ductus to close (Post-natal).
Comment: Continuous “Machinery” murmur. Indomethacin helps close it; Prostaglandins keep it open.
Shunt Echo Congenital PDA
ALCAPA Connections: Anomalous Left Coronary Artery from Pulmonary Artery.
Physiology: “Steal” phenomenon (blood flows from coronary into PA).
Comment: Causes infant myocardial infarction/ischemia. Bland-White-Garland syndrome.
Coronary Anomaly Angio/CT Congenital ALCAPA
Anomalous Origin (High Risk) Position: Coronary artery coursing *between* the Aorta and Pulmonary Artery (Interarterial).
Comment: High risk of sudden death in young athletes due to compression during exercise.
Coronary Anomaly CT Angio Congenital Coronary Anomalies

 

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
ARVC Inherited Cardiomyopathy Arrhythmogenic RV Dysplasia Definition: Genetic defect in desmosomes leading to fibro-fatty replacement of the RV myocardium.
Comment: Major cause of sudden cardiac death in young athletes. “Triangle of Dysplasia” involves Inflow, Outflow, and Apex.
MRI: Fatty infiltration, wall thinning, and aneurysms
Function: RV dilation and global/regional dysfunction
Epsilon Wave: Pathognomonic EKG finding
Right Ventricle Inherited ARVC
Dilated Cardiomyopathy (Familial) Inherited Cardiomyopathy Familial DCM Definition: Genetic form of DCM (often cytoskeletal protein mutations like Lamin A/C or Titin).
Comment: Accounts for 20-35% of all DCM cases. Indistinguishable from idiopathic DCM on imaging without family history.
Size: Globular LV enlargement
Function: Systolic failure (Reduced EF)
MRI: Mid-wall septal fibrosis (LGE) is common in Lamin A/C mutations
Left Ventricle Inherited Dilated Cardiomyopathy Case 007H
Hypertrophic Cardiomyopathy (HCM) Inherited Cardiomyopathy HOCM / IHSS Definition: Autosomal dominant disorder of the sarcomere causing inappropriate hypertrophy (>15mm) without a hemodynamic cause.
Comment: Asymmetric Septal Hypertrophy (ASH) is the classic pattern. Risk of obstruction (HOCM) and sudden death.
Shape: Asymmetric septal thickening (>1.5x posterior wall)
Motion: Systolic Anterior Motion (SAM) of Mitral Valve
MRI: Patchy LGE at RV insertion points
Left Ventricle Inherited Hypertrophic CM Case 068H
LV Non-Compaction Inherited Cardiomyopathy Spongy Myocardium Definition: Arrest of normal myocardial compaction during weeks 5-8 of embryology.
Comment: Results in a two-layered myocardium: a thin compacted epicardial layer and a thick non-compacted endocardial layer.
Character: Deep trabecular recesses communicating with the cavity
Ratio: Non-compacted to Compacted ratio > 2.3 (in diastole)
Location: Apex and Lateral wall
Left Ventricle Inherited Non-Compaction
Marfan Syndrome Connective Tissue Disorder Fibrillin-1 Mutation Definition: Autosomal dominant connective tissue disorder affecting the skeletal, ocular, and cardiovascular systems.
Comment: Cardiovascular mortality is due to aortic root dilation, dissection, or rupture.
Aorta: “Tulip Bulb” root aneurysm (Annuloaortic Ectasia)
Valve: Mitral Valve Prolapse (MVP) and Aortic Regurgitation
Chest: Pectus excavatum / carinatum
Aorta / Valves Inherited Marfan Syndrome
Noonan Syndrome Genetic Syndrome Male Turner Syndrome Definition: Autosomal dominant disorder (RAS-MAPK pathway) associated with short stature and heart defects.
Comment: The cardiac phenotype is often Pulmonary Stenosis (dysplastic valve) and/or Hypertrophic Cardiomyopathy.
Valve: Pulmonary Stenosis (thickened, dysplastic leaflets)
Myocardium: Concentric or Asymmetric Hypertrophy (mimics HCM)
Pulmonary Valve Inherited Pulmonary Stenosis

 

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Amyloidosis (Cardiac) Protein Infiltration Stiff Heart Syndrome
ATTR / AL Amyloid
Definition: Infiltration of the myocardial interstitium by insoluble amyloid fibrils.
Comment: The classic cause of Restrictive Cardiomyopathy. T1 mapping on MRI is diagnostic (high native T1).
MRI: Diffuse subendocardial LGE; inability to null myocardium
Size: Concentric thickening of LV and RV
Atria: Bi-atrial enlargement
Myocardium Infiltrative Faces of Amyloidosis Case 006H
Endomyocardial Fibrosis Fibrotic Infiltration Löffler’s Endocarditis Definition: Infiltration by eosinophils (Löffler’s) leading to dense apical fibrosis and thrombosis.
Comment: A major cause of restrictive cardiomyopathy in tropical regions. Causes “Apical Obliteration.”
Shape: Obliteration of the LV/RV apex by thrombus/fibrosis
Function: Restrictive filling pattern
MRI: Apical subendocardial LGE
Ventricles (Apices) Infiltrative Restrictive CM (EMF)
Hemochromatosis Iron Infiltration Iron Overload Definition: Infiltration of cardiomyocytes by iron, causing toxicity.
Comment: Reversible cause of heart failure. Leads to Dilated or Restrictive physiology.
MRI (T2*): Low signal intensity (dark myocardium) proportional to iron load
Liver: Associated dark liver
Function: Diastolic dysfunction (early)
Myocardium Infiltrative Hemochromatosis Case 41M
Lipomatous Hypertrophy Fatty Infiltration Lipoma of IAS Definition: Infiltration of the Interatrial Septum by non-encapsulated fat.
Comment: Benign and usually incidental. Distinguished from Lipoma by its “Dumbbell” shape (sparing the Fossa Ovalis).
Character: Fat density (-100 HU) or high T1 signal
Shape: Bilobed or Dumbbell shape
Position: Interatrial Septum
Interatrial Septum Infiltrative Fat in Atrial Septum
Sarcoidosis (Cardiac) Granulomatous Infiltration Cardiac Sarcoid Definition: Infiltration of the myocardium by non-caseating granulomas.
Comment: “The Great Imitator.” Can cause arrhythmias (heart block/VT) and heart failure.
MRI: Patchy, non-vascular distribution of LGE (Basal Septum is common)
PET: Focal FDG uptake (active inflammation)
Nodes: Hilar adenopathy
Myocardium Infiltrative Cardiac Sarcoidosis Case 016H

 

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Anthracycline Toxicity Drug Toxicity Doxorubicin Cardiomyopathy
Chemo Heart
Definition: Dose-dependent, cumulative myocardial damage caused by chemotherapeutic agents (e.g., Adriamycin).
Comment: Causes irreversible myocardial cell death and fibrosis.
Function: Global hypokinesis and reduced EF
Shape: Dilated Cardiomyopathy phenotype
MRI: Diffuse interstitial fibrosis (High T1)
Myocardium Iatrogenic Dilated Cardiomyopathy
Catheter/Lead Perforation Procedural Complication Cardiac Perforation Definition: Penetration of the myocardial wall by a pacemaker lead, wire, or catheter.
Comment: The RV apex is the most common site due to the thin wall.
Position: Lead tip extends beyond the epicardial fat border
Effusion: Hemopericardium / Tamponade
CT: Tip seen within extracardiac fat or liver
RV Wall Iatrogenic Pacemaker Complications
Post-Pericardiotomy Syndrome Post-Surgical Dressler’s Syndrome Definition: Inflammatory pericarditis occurring days to weeks after cardiac surgery (CABG/Valve).
Comment: Autoimmune reaction to pericardial injury.
Effusion: Loculated or free pericardial fluid
Enhancement: Thickened pericardial layers
Pleura: Associated pleural effusions
Pericardium Iatrogenic Post-Pericardiotomy Syndrome
Pulmonary Vein Stenosis Post-Ablation PV Stenosis Definition: Acquired narrowing of the pulmonary vein ostia following Radiofrequency Ablation (RFA) for Atrial Fibrillation.
Comment: Causes localized pulmonary venous hypertension in the affected lobe.
Size: Focal constriction at the veno-atrial junction
Lung: Focal ground-glass opacity (edema) in the drained lobe
Pulmonary Veins Iatrogenic Pulmonary Vein Stenosis
Radiation Heart Disease Therapy Related Radiation Induced Heart Disease Definition: Spectrum of cardiac damage resulting from thoracic radiation (Breast Ca, Lymphoma).
Comment: Manifests 10-20 years post-treatment. Fibrosis is the hallmark.
Valve: Fibrotic thickening/calcification (Aortic/Mitral)
Aorta: “Porcelain Aorta” (circumferential calcification)
Coronary: Ostial stenosis
Valves / Pericardium Iatrogenic Radiation Heart Disease

 

Disease / Diagnosis Grouped Category Alternative Names Definition & Comment Imaging Features Structure Category Link to TCV Page Linkable Case Examples
Idiopathic Dilated CM Primary Cardiomyopathy DCM Definition: Dilation and impaired contraction of the left or both ventricles with no identifiable cause (after ruling out CAD, Alcohol, Viral).
Comment: Diagnosis of exclusion. Represents ~50% of all DCM cases.
Size: Globular enlargement (LVIDd > 5.8cm)
MRI: Absence of LGE (or mid-wall septal streak)
Function: Reduced EF (< 40%)
Ventricles Idiopathic Dilated Cardiomyopathy Case 007H
Idiopathic Pulmonary HTN Pulmonary Vascular IPAH / Primary PHTN Definition: Elevated pulmonary artery pressure (mPAP > 20 mmHg) with no identifiable secondary cause (Lung, Left Heart, or TE disease).
Comment: A diagnosis of exclusion affecting the pulmonary arterioles (plexiform lesions).
Size: Dilated MPA (> 29mm)
RV: Hypertrophy and Dilation
Lung: Clear parenchyma (“Pruning” of distal vessels)
Pulmonary Artery Idiopathic Pulmonary Hypertension
Idiopathic Restrictive CM Primary Cardiomyopathy Primary Restrictive CM Definition: Restrictive filling pattern with normal wall thickness and systolic function, without infiltrative disease.
Comment: Rare. Must exclude Amyloidosis and Sarcoidosis.
Atria: Massive Bi-atrial enlargement
Ventricles: Normal size and thickness
Function: Severe diastolic dysfunction (Grade III/IV)
Myocardium Idiopathic Restrictive CM

TCV

🠉
🠋