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	Cardiomegaly in large VSD	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	Calcification score	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) Rheumatic fever Endocarditis	Dyspnea on exertion Paroxysmal nocturnal dyspnea Orthopnea New onset atrial fibrillation	Mitral facies Heart murmur Jugular vein distension 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)	P mitrale Atrial fibrillation: No P waves and irregularly irregular rhythm Right axis deviation 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 Splaying of subcarinal angle (>120 degrees) Calcification of mitral valve Kerley B lines	Reduced valve leaflet mobility Valve calcification Doming of mitral valve Valve thickening Enlargement of left atrium	Right heart catheterization: Pulmonary capillary wedge pressure (left atrial pressure) 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)	Hemoptysis (heart failure) Ortner's syndrome
Mitral Regurgitation	CAD MI Rheumatic fever Endocarditis Mitral valve prolapse Cardiomyopathy Radiation therapy Trauma	Palpitations 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	Holosystolic 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 Decrease in intensity on standing or valsalva maneuver	P mitrale in lead II Increased QRS voltage Right axis deviation Atrial fibrillation	Acute MR Kerley B lines No enlargement of cardiac silhouette 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	Decompensated and acute MR may lead to heart failure
Atrial septal defect	Frequent respiratory or lung infections Dyspnea Tiring when feeding (Infants) Shortness of breath on exertion Palpitations Swelling of feet	Shortness of breath Fatigue Failure to thrive Swelling of feet and abdomen (Right heart failure) Palpitations Respiratory infections	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	Normal Prolonged PR interval Right bundle branch block ECG findings varies according to the underlying type of ASD	Increased pulmonary markings Cardiomegaly Triangular appearance of heart Schimitar sign	Gold standard test for diagnosis of atrial septal defect (for more information click Atrial septal defect echocardiography)	Defect size Pulmonary venous return Pulmonary vascular resistance Pulmonary artery hypertension	Asymptomatic until later part of their life May be associated with migraine with aura
Left Atrial Myxoma	Dyspnea Orthopnea Pulmonary edema Hyperpigmentation of skin and endocrine activity Cerebral embolism	Symptoms may mimic mitral stenosis	Skin Signs of an embolic phenomenon Raynaud's phenomenon Swelling Clubbing 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.,	Initial and most useful diagnostic study For more information click Myxoma echocardiography or ultrasound	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	Dyspnea on exertion Recent onset of congestive heart failure	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 Right axis deviation Right atrial enlargement Right ventricular hypertrophy	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