Sandbox: Ifeoma

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Classification of dextrocardia

Dextrocardia Types Description
Dextrocardia with situs solitus
  • Dextrocardia with normally related great arteries and D-transposition (complete transposition) or L-transposition (congenitally corrected transposition) of the great arteries. Some examples include dextrocardia with D-loop ventricles and normally related great arteries, with L-loop ventricles and L-TGA (congenitally corrected TGA).
  • Embryologic failure of the final leftward shift of the ventricles during development results in dextrocardia with situs solitus, D-loop ventricles, and normally related great arteries.
Dextrocardia with situs inversus
  • May present with dextrocardia with inversely related great arteries and D-transposition (congenitally corrected transposition) or L-transposition (“uncorrected” transposition) of the great arteries. An example is dextrocardia with D-loop ventricles and D-TGA (congenitally corrected TGA).
Dextrocardia with situs ambiguous (either polyspenia or asplenia)
  • Dextrocardia with any of the above relationships between the ventricles and great vessels.
Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Imaging
Exertional dyspnea Failure to thrive Recurrent respiratory infections Murmur on auscultation Peripheral edema Clubbing Echocardiography Chest x-ray Cardiac CT
Patent foramen ovale
  • Appearance of at least 3 micro-bubbles in the left atrium within three cardiac cycles after complete opacification of the right atrium
Non specific
  • A contrast agent jet from the left atrium to the right atrium toward the inferior vena cava with channel-like appearance of the interatrial septum
  • Echocardiogram
  • It is associated with paradoxical embolism, migraine headache, and decompression sickness in divers
Atrial septal defect +/− +/− +/−
  • Systolic flow murmur in the upper left sternal border
  • Wide, fixed splitting of S2
  • Diastolic flow rumble across the tricuspid valve
+/− +/−
  • Hypermobile interatrial septum
  • Abrupt septal irregularity
  • Right atrial and ventricular volume overload
  • Pulmonary artery dilatation
  • Cardiomegaly
  • Pulmonary artery enlargement/increased pulmonary vascularity
  • Enlargement of the right atrium and ventricle
  • Echocardiogram
  • Atrial septal defect is classified into 5 types including ostium primum defect, ostium secundum defect, superior sinus venosus defect, inferior sinus venosus defect, and coronary sinus defect
Ventricular septal defect -/+ -/+ After Eisenmenger syndrome
  • Holosystolic murmur
  • May mimic aortic stenosis(mid/end dyastolic murmur due to increased pulmonary circulation)
-/+ -/+
  • Defect localization
  • septal dropout in the area adjacent to the tricuspid septal leaflet and below the right border of the aortic annulus
  • Direction of jet
  • Direct visualisation of murmur
  • Echocardiogram
Diseases Exertional dyspnea Failure to thrive Recurrent respiratory infections Murmur on auscultation Peripheral edema Clubbing Echocardiography Chest x-ray Cardiac CT Gold standard Additional findings
Patent ductus arteriosus
  • Not at beginning
  • May be produced during the course of disease
  • Depends on the size
-
  • Continuous machine-like murmur
-
  • May be present by progressing
  • Golden standard
  • In color-Doppler visualization of flow through the patent duct which has a high velocity
  • Non-specific
  • Used for determining Krichenko classification
  • Echocardiogram
  • Krichenko criteria for classification is a very important factor for treatment
Coarctation of the aorta +/− +/−
  • Systolic murmur over the upper sternal border with radiation to the back
  • Murmur of mitral regurgitation (holosystolic murmur best heard at the apex)
  • Narrowing of the aortic arch at the level of the isthmus
  • Left ventricular hypertrophy
  • Notching of the posterior fourth to eighth ribs due to dilated intercostal arteries
  • Indentation of the aorta at the site of coarctation with pre- and post-stenotic dilation of the aorta (classic "3 sign")
  • Dilation of the intercostal arteries
  • Echocardiogram
  • Patients present with arm-leg blood pressure gradient of >20mmHg
Aortic stenosis + + +
  • Crescendo-decrescendo mid-systolic (or ejection systolic) murmur
+/-
  • Depending on severity
  • Used for finding the location of stenosis
  • Finding severity
  • Evaluating the flow jet with color-Doppler ultrasound technique
  • Non-specific at the beginning
  • At progressed stage calcification of the valve and cardiomegally
  • MRI
  • MRI provides a more detailed structural and dynamic assessment of the aortic valve and left ventricle, in particular
Pulmonary stenosis
  • Depending on severity
- -/+
  • Continuous systolic murmur
- -/+
  • Right atrial hypertrophy
  • Non-specific
  • Direct visualization of stenosis
  • Echocardiogram






Diseases Pathophysiology Shunt Symptoms Diagnosis Echocardiography findings Physical examination Treatment Complications
Patent foramen ovale
  • Failure of fusion of the septum primum and septum secundum leading to a flap valve opening.
  • Right-to-left shunt
  • More prominent with increased right atrial pressure.
  • Majority of patients are asymptomatic
  • TEE (Gold standard)
  • TTE
  • TCD
  • Appearance of at least 3 micro-bubbles in the left atrium within three cardiac cycles after the complete opacification of the right atrium
  • Percutaneous closure
  • Anticoagulants
  • Antiplatelets
  • Paradoxical embolism
  • Migraine with aura
  • Decompression sickness in divers
  • Platypnea-orthodeoxia syndrome
Atrial septal defect
  • Ostium secundum defect: Failure of the septum secundum to occlude the ostium secundum.
  • Ostium primum defect: Failure of the ostium primum to fuse with the endocardial cushions.
  • Superior sinus venosus defect: The orifice of the superior vena cava overrides the atrial septum above the fossa ovalis.
  • Inferior sinus venosus defect: The orifice of the inferior vena cava overrides the left and right atrium.
  • Coronary sinus defect: Absence of a portion of the common wall that separates the coronary sinus and the left atrium.
  • Continuous left-to-right shunt
  • Failure to thrive, tachypnea, recurrent respiratory infections, heart failure
  • Commonly asymptomatic during childhood and adolescence
  • Adults with large shunts may become symptomatic in the fourth decade presenting with fatigue, exercise intolerance, palpitations, syncope, and shortness of breath.
  • TTE (Gold standard)
  • Cardiac CT
  • Cardiac MRI
  • Hypermobile interatrial septum
  • Abrupt septal irregularity
  • Right atrial and ventricular volume overload
  • Pulmonary artery dilation
  • Coronary sinus defect: Enlarged ostium of the coronary sinus and unroofing of the terminal portion of the coronary sinus
  • Systolic flow murmur in the pulmonary valve region
  • Wide, fixed splitting of S2
  • Diastolic flow rumble across the tricuspid valve
  • Right ventricular heave
  • Spontaneous closure
  • Percutaneous transcatheter closure
  • Surgical closure
  • Right sided heart failure
  • Peripheral edema
  • Eisenmenger syndrome (cyanosis)
  • Paradoxical emboli
  • Pulmonary hypertension
Pulmonary ateriovenous fistula
  • Abnormal blood vessel(s) connecting the pulmonary arteries and veins directly without interposition of pulmonary capillaries
  • Right-to-left shunt between the pulmonary artery and pulmonary vein
  • Symptoms may occur only after the second decade
  • Cyanosis
  • Hemoptysis
  • Chest CT
  • Pulmonary arteriogram
  • Appearance of contrast bubbles in the left atrium three to five cardiac cycles after appearance in the right atrium
  • Clubbing
  • Systolic/continuous murmur
  • Embolization
  • Surgical resection
  • Cerebral ischemia/abscess
  • Hemothorax

Differential table for aortic stenosis

Diseases History Symptoms Physical Examination Murmur Diagnosis Other Findings
ECG CXR Echocardiogram Cardiac Catheterization
Aortic stenosis
  • Age (aortic valve calcification)
  • Syncope
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Acute rheumatic fever
  • Chest pain
  • Dyspnea on exertion
  • Palpitations
  • Symptoms of heart failure
  • Pulsus parvus et tardus
  • Pulmonary rales
  • Peripheral edema (In CHF patients)
  • Jugular venous distension
  • Enlarged and laterally displaced point of maximal impulse
  • Crescendo-decrescendo systolic murmur
  • Best heard at the right upper sternal border
  • Radiation to the carotid arteries
  • Increases with squatting
  • Decreases with valsalva maneuver
Left ventricular hypertrophy:
  • Wide QRS complex (especially in leads V1-V6)
  • ST depression in leads V5-V6
  • Left axis deviation
  • Enlarged left ventricle
  • Enlarged left atrium and pulmonary artery in severe cases
  • Calcification of the aortic valve
  • Thickening and calcification of the aortic valve
  • Left ventricular hypertrophy
Left heart catheterization:
  • Left ventricular and aortic pressures
  • The left ventricle generates higher pressures than what is transmitted to the aorta
  • Associated with von Willibrand disease
Mitral Stenosis
  • Age ( Mitral annular calcification in older patients)
  • Mitral facies
  • Heart murmur
  • Apical impulse displaced laterally or not palpable
  • Diastolic thrill at the apex
  • Signs of heart failure in severe cases
  • Diastolic murmur
  • Low pitched
  • Opening snap followed by decrescendo-crescendo rumbling murmur
  • Best heard with the bell of the stethoscope at apex at end-expiration in left lateral decubitus position
  • Intensity increases after a valsalva maneuver, after exercise and after increased after load (eg., squatting, isometric hand grip)
  • Right ventricular hypertropy: Dominant R wave in V1 and V2
  • Straightening of the left border of the heart suggestive of enlargement of the left atrium
  • Double right heart border (Enlarged left atrium and normal right atrium)
  • Prominent left atrial appendage
  • Reduced valve leaflet mobility
  • Valve calcification
  • Doming of mitral valve
  • Valve thickening
  • Enlargement of left atrium
Right heart catheterization:

Left heart catheterization:

  • Pressures in left ventricle
  • Determines the gradient between the left and right atrium during ventricular diastole (marker of the severity of mitral stenosis)
Mitral Regurgitation
  • Trauma
  • Symptoms of heart failure in severe cases
Palpation
  • Brisk carotid upstroke and hyperdymanic carotid impulse on palpation
  • Apical impulse is displaced to left
  • S3 and a palpable thrill

Auscultation

  • Murmur
  • High pitched, blowing
  • Radiates to axilla
  • Best heard with the diaphragm of the stethoscope at apex in left lateral decubitus position
  • Intensity increases with hand grip or squatting
Acute MR

Chronic MR

  • Enlarged cardiac silhouette
  • Straightening of left heart border
  • Splaying of subcarinal angle
  • Calcification of mitral annulus
  • Double right heart border
  • Enlargement of left atrium and ventricle
  • Identify valve abnormality
  • Valve calcification
  • Severity of regurgitation
  • Grading of MR is done with left ventriculography
Atrial septal defect
  • Frequent respiratory or lung infections
  • Dyspnea
  • Tiring when feeding (Infants)
  • Shortness of breath on exertion
  • Palpitations
  • Swelling of feet
Inspection
  • Precordial bulge
  • Precordial lift

Palpation

  • Right ventricular impulse
  • Pulmonary artery pulsations
  • Thrill

Auscultation

  • Murmur
  • Midsystolic (ejection systolic) murmur
  • Widely split, fixed S2
  • Upper left sternal border
  • Increased pulmonary markings
  • Cardiomegaly
  • Triangular appearance of heart
  • Schimitar sign
Left Atrial Myxoma
  • Symptoms may mimic mitral stenosis
Skin

Auscultation:

  • Lung: Fine crepitations
  • Heart: Characteristic "tumor plop"
  • Early diastolic sound as "tumor plop"
  • Low frequency diastolic murmur may be heard if the tumor obstructing mitral valve
  • Often normal
  • Often normal

Rare findings:

  • cardiomegaly
  • Left atrial enlargement
  • tumor calcification etc.,
  • Useful to detect vascular supply of the tumor by the coronary arteries
  • Associated with Carney complex (genetic predisposition)
Prosthetic Valve Obstruction
  • History of valve replacement
  • Systemic embolism
  • Shortness of breath
  • Fatigue
Ausculation

Muffling of murmur

  • Muffling or disappearance of prosthetic sounds
  • Appearance of new regurgitant or obstructive murmur
  • Degree of stenosis
  • Assess thrombus size and location
  • Differentiate between thrombus, pannus and vegetations
Causes:
  • Thrombus
  • Pannus formation
Cor Triatriatum
  • Dsypnea on exertion
  • Orthopnea
  • Tachypnea
  • Palpitations
  • Growth failure
Auscultation
  • Murmur

Other findings

  • Signs of heart failure
  • Diastolic murmur with loud P2
  • No opening snap or a loud S1
Non specific but may have
  • Normal cardiac silhouette
  • Hemodynamic changes similar to mitral stenosis (non specific findings)
  • Direct visualization of membrane through the atrium
  • +/- visualization of accessory chamber
  • Normal left ventricular hemodynamic profile with a trans atrial gradient
Types
  • Cor triatriatum sinistrum
  • Cor triatriatum dextrum
Congenital Mitral Stenosis
  • Respiratory distress shortly after birth
  • Recurrent severe pulmonary infections
  • Other associated congenital cardiovascular anamolies
  • Atrial fibrillation

Infants:

  • Exhaustion and sweating on feeding
  • Rapid breathing
  • Failure to thrive
  • Pulmonary infections
  • Chronic cough

Older patients:

  • Dyspnea
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Peripheral edema
  • Fatigue
Auscultation
  • Murmur

Other findings

  • Signs of heart failure
Mild-Moderate
  • Loud S1
  • Loud P2
  • Low frequency diastolic murmur best heard at the apex

Severe

  • Soft S1
  • Loud pulmonic component of S2 with minimal respiratory splitting of S2
  • Holodiastolic murmur with presystolic accentuation best heard at the apex
  • Early diastolic murmur of pulmonic valve regurgitation
  • Sharp P waves in leads I and II
  • Inversion of P wave in lead III
  • Marked Q waves in leads II and III
  • Left atrial dilation
  • Moderate enlargement of right heart
  • Pulmonary venous congestion
  • Esophageal compression
  • Reduced valve leaflet mobility
  • Left atrial size
  • Severity of mitral stenosis
Very rare condition
Supravalvular Ring Mitral Stenosis
  • Other associated congenital heart defects
  • Fatigue
  • Frequent respiratory infections
  • Failure to thrive
  • Poor feeding
  • Precocious congestive heart failure
  • Shortness of breath
  • Tachypnea
  • Dyspnea
  • Nocturnal cough
  • Heamoptysis
  • Syncope
Auscultation:

Lungs: Fine, crepitant rales and rhonchi or wheezes may be present

Heart: Murmur

  • An apical mid diastolic murmur with presystolic accentuation
  • No opening snap
  • The murmur is more prominent if associated with VSD or PDA
  • Left atrial and ventricular enlargement
  • Alveolar edema
Supramitral ring:
  • Associated with normal mitral valve apparatus

Intramitral ring:

  • Hypomobility of the posterior leaflet
  • Reduced interpapillary muscle distance
  • Reduced chordal length
  • Dominant papillary muscle
  • Hypoplastic mitral annulus

(Difficult to visualize membrane <1mm in size)

  • Persistently elevated pulmonary venous pressures
  • Increased pulmonary artery pressure
Types
  • Supramitral
  • Intramitral

It is attached between the opening of the atrial appendage and the mitral annulus which helps in differentiating with Cor triatriatum sinister.

  • Intramitral type is associated with shone complex