Heart valve disease, congenital heart disease · mitral regurgitation, anemia, hyperthyroidism,...

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Heart valve disease

Congenital heart disease

Kristína Repová, M.D., PhD.

[email protected]

Institute of Pathophysiology, Faculty of Medicine, Bratislava

The heart

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Phases of the cardiac cycle

https://www.artstation.com/artwork/ZLrON

Heart valves

• AV valves:

• Tricuspid valve (TriV) between RA and RV

• Mitral valve (MiV) between LA and LV

• open during diastole

• close during systole

• Semilunar valves:

• Pulmonic valve (PuV) between RV and pulmonary artery

• Aortic valve (AoV) between LV and aorta

• open during systole

• close during diastole

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Heart sounds• S1:

• closure of MiV and TriV

• duller, lower-frequency sound, in the beginning of systole

Normal

Loud S1: mitral stenosis, ejection sound masquerading as S1

Soft or absent S1: 1° atrioventricular block, aortic regurgitation: acute (from preclosure of mitral valve)

• S2:

• closure of AoV and PuV

• sharper, louder sound, end of the systole, beginning of diastole

Normal / physiologic split during inspiration (dalayed PuVclosure)

Loud S2: dilated aorta, dilated pulmonary artery

Soft or absent S2: aortic stenosis, pulmonic stenosis, chronic obstructive pulmonary disease

Heart sounds• S3 (S1....S2..S3 = gallop sound):

• inflowing blood against a distended or incompliant ventricle in early diastole

• often a sign of heart failure

• low-frequency sound, occurs cca. 120-150 msec after S2

Normal: high cardiac output (athletes, pregnancy)

mitral regurgitation, anemia, hyperthyroidism, hypertrophic cardiomyopathy, constrictive pericarditis, myxoma

• S4:• forceful atrial contraction during late diastole that ejects

blood into a stiff ventricle

• low-frequency gallop sound, occurs ~90 msec before S1

Left ventricular hypertrophy (LVH):

• hypertension, aortic stenosis, hypertrophiccardiomyopathy

Right ventricular hypertrophy (RVH):

• pulmonic stenosis, pulmonary arterial hypertension

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Heart defects• Developmental defects and acquired changes in heart morphology, interfere with

pumping function of the heart

• Functional consequences:

• Left-to-right shunt

• blood volume through pulmonary circulation > peripheral circulation

ventricular septal defect, patent ductus arteriosus, atrial septal defect, atrioventricular defect

• Right-to-left shunt

• blood volume through pulmonary circulation < peripheral circulation

• arterial blood from lungs is mixed with venous blood

immediately after birth: Fallot's tetralogy, pulmonary valve atresia, tricuspidatresia

• Stenosis

• narrowing of a part of heart

• to pump blood through the narrowing requires higher pressure

• Regurgitation

• valvular insufficiency

• part of the blood moves opposite direction during the heart cycle

Heart valve disease

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Acquired heart defects

• Mitral stenosis

• Mitral insufficiency (regurgitation)

• Mitral valve prolapse

• Aortic stenosis

• Aortic insufficiency (regurgitation)

• Tricuspid stenosis

• Tricuspid insufficiency (regurgitation)

• Pulmonary stenosis

• Pulmonary insufficiency (regurgitation)

Acquired heart defects

• Etiology:

• rheumatic heart disease

• endocarditis

• degenerative changes: fibrotization or calcification

• secondary dilation in cardiomyopathies, dysfunction of papilary muscles

• Usually combination of stenosis and insufficiency

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Rheumatic heart disease

repeated group A streptococcus (GAS) infections (pharyngitis)

molecular mimicry between GAS antigens and host tissues

exaggerated B- and T-cell mediated immune response

1st episode of acute rheumatic fever (ARF)

repeated GAS infections and ARF

Rheumatic heart disease:

pancarditis

valvular damage: mitral

regurgitation � stenosis

Brain:

Sydenham's

chorea

Joints:

polyarthritis

Skin:

erythema marginatum

subcutaneous nodules

(Osler`s nodes)

Mitral valve (MiV)

Normal mitral valve area: 4-6 cm2

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Mitral stenosis (MiS)

• Mitral stenosis



• Aortic stenosis






Mitral stenosis

• symptoms begin at areas < 2 cm2

• Fibrotic/calcific thickening, contractures and adhesions of both leaflets (cusps), chordae tendineae fuse and shorten

Etiology:

• several years (10-20) after rheumatic fever

• severe mitral annular calcification

• SLE

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Mitral stenosis - pathophysiology

� LA pressure

LA hypertrophy, dilation

atrial fibrillation

thrombi

emboli

� pressure in pulmonary

capillaries

pulmonary hypertension

pulmonary

edema

hemoptysis

� CORV dilation

tricuspid regurgitation

(tricuspidalization) � Sp.

peripheral

vasoconstriction

� LV

contractilityfailure of

right heart

Mitral stenosis - symptoms• (Exertional) dyspnea

• Atrial fibrillation:

• � LA pressure → � LA wall tension → � energy consumption → Na-K-ATPase failure → ectopic region(s) → atrial extrasystole, fibrillation

• Impaired LV filling, tachyarrhytmia, blood stagnation in dilated LA, thrombi

• Embolisation:

• Brain, lungs, extremities, kidneys, …

• Hemoptysis:

• rupture of bronchial vessels due to elevated pulmonary pressure

• Cyanosis:

• Peripheral, compensatory peripheral vasoconstriction

• Mitral facies (malar flush): cutaneous vasodilation, chronic hypoxemia, � CO2, pinkish-purple patches on the cheeks

• Fatigue, weakness: � CO

• Heart failure, pulmonary edema:

• due to tachycardia, infection, progressive mitral stenosis

• Normal or slightly low systemic arterial blood pressure

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Mitral stenosis - symptoms

Mitral stenosis – auscultation• Accentuated and slightly delayed S1:

• rapid closing of MiV during isometric contraction

• strong contraction of LV (due to Sp.)

• Opening mitral snap:

• rapid initial opening of MiV and rapid blood flow to LV (due to � AV gradient)

• Diastolic murmur:

• low-pitched diastolic rumble most prominent at the apex

• heard best with the patient lying on the left side in held exspiration

• intensity of the diastolic murmur does not correlate with the severity of the stenosis

• Pulsus parvus (small amplitude)

http://www.practicalclinicalskills.com/auscultation-repetition-training-reference.aspx?caseID=97

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Mitral stenosis – evaluation• ECG:

• may show atrial fibrillation and LA enlargement (P-mitrale)

• CXR:

• LA enlargement, small LV and pulmonary congestion

• Occasionally calcified MV

• ECHO:

• The GOLD STANDARD for diagnosis

• Asses mitral valve mobility, gradient and mitral valve area

Mitral stenosis – worse prognosis

• worsening of stenosis

• exercise, atrial fibrillation, tachycardia, fever, severe anemia, thyreotoxicosis:

• � frequency → relative shortening of diastole → impaired LV filling → � CO

• pregnancy:

• � circulating blood volume

• infections:

• � need of tissues for O2

• Mortality: Due to progressive pulmonary congestion, infection and thromboembolism

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Mitral stenosis – therapy

• Medications:

• MiS is a mechanical problem and medical therapy does not prevent progression

• β-blockers, CCBs, digoxin: control heart rate and hence prolong diastole for improved diastolic filling

• Diuretics for fluid overload

• Warfarin: in dilated LA

• Surgery:• percutaneous mitral balloon valvotomy• mitral valve replacement

Mitral insufficiency (MiI)

• Mitral stenosis



• Aortic stenosis






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Mitral insufficiencyEtiology:

• Acute MiI:

• papillary muscle rupture – after acute MI

• blunt chest wall trauma

• infective endocarditis (IE): the leaflets and chordae shrink, thicken, and become more rigid → impaired valve closure

• chordal rupture / leaflet flail – mitral valve prolaps (MVP), IE

• Chronic MiI:

• rheumatic fever

• mitral valve prolapse (Barlow’s syndrome): the chordae are too long and the leaflets thus bulge like a parachute into the LA, where they open

• myxomatous

• mitral annular calcification

• endocarditis (healed)

• congenital – cleft

• hypertrophic obstructive cardiomyopathy

• dilated cardiomyopathy

• ischemic – LV remodeling

Mitral insufficiency – pathophysiology

• during systole some of the blood in the LV flows back

(“regurgitates”) into the LA → � volume load on the left

heart

• regurgitant volume may be 80% of the SV, depends on:

• the mitral opening area in systole

• the pressure gradient from LV to LA during ventricular systole

• the duration of systole

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Mitral insufficiency – pathophysiology

Chronic

� atrial compliance

volume

overload

atrial dilation

atrial damage

tachyarrhythmia

(atrial fibrillation)

�� regurgitant

volume

left heart failure

Acute

� atrial compliance

� LA pressure

dyspnea, hemoptysis

pulmonary edema

pulmonary

hypertension

right heart failure

� forward CO

� systolic BP

tachycardia

�� regurgitant

volume

�� mitral

regurgitation

ventricular dilation

� mitral

regurgitation

Mitral insufficiency – symptoms• Mild-moderate MiI: asymptomatic

• Chronic severe MiI: fatigue, exertional dyspnea (� CO, pulmonary edema)

• Palpitations: excessive volume, AF

• Pulmonary edema, dyspnea

• Dilation of right heart � tricuspidalization, pulmonary insufficiency

• Low LV-filling:

• low cardiac output

• sympathoactivation

• tachycardia

• vasoconstriction

• cyanosis

• fatigue

• (hemoptoe, embolization, cyanosis, fibrillation)

• BP normal

• Arterial pulse: sharp upstroke

• Systolic thrill palpable at the apex

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Mitral insufficiency - symptoms

Mitral insufficiency – auscultation

• S1 absent, soft:

• incomplete closing of MiV in systole

• Low-pitched S3:

• large volume of blood returning to the LV in early diastole

• sudden tensing of the papillary muscles, chordae tendineae and valve leaflets in acute MiI

• Holosystolic murmur:

• loudest at the apex with radiation to the axilla and left infrascapulararea

• constant in intensity, blowing, high-pitched


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Mitral insufficiency - evaluation• ECG: LA enlargement, atrial fibrillation and LV hypertrophy

with severe MR

• CXR: LA, LV enlargement, central pulmonary artery enlargement

• ECHO: Estimation of LA, LV size and function, valve structure assessment

Mitral insufficiency – worse prognosis

• additional aortic stenosis or hypertension

• tachycardia: physical activity, tachyarrhythmia

→ � LA pressure

• infections

• repeated attacks of rheumatic carditis

• Mortality: from progressive dyspnea and heart failure

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Mitral insufficiency - treatment

• Medications:

• Vasodilator: hydralazine

• Rate control for atrial fibrillation with β-blockers, CCB, digoxin

• Do not attempt to alleviate tachycardia with beta-blockers in acute MiI. Mild-to-moderate tachycardia is beneficial in these patients because it allows less time for the heart to have backfill, which lowers regurgitant volume

• Anticoagulation in atrial fibrillation and flutter

• Diuretics for fluid overload

• Surgery:

• MiV repair

• MiV replacement

Mitral valve prolapse (MiVP)

• Mitral stenosis



• Aortic stenosis






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Mitral valve prolapse

= systolic click-murmur syndrome, Barlow's syndrome

• displacement of an abnormally thickened MiV leaflet into the LA during systole

• common, but variable

• inherited: collagen disorder, Marfan syndrome, osteogenesisimperfecta

• myxomatous degeneration of MiV leaflets, dilated annulus, elongated or ruptured chordae

� mitral regurgitation

Aortic valve (AoV)

Normal aortic valve area: 3,5 cm2

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Aortic stenosis (AoS)

• Mitral stenosis



• Aortic stenosis






Aortic stenosis

• 25% of all patients with chronic valvular defects

• severe: AoV area < 1cm2

Etiology:

• rheumatic fever

• sclerotic degeneration / calcification of AoV

• bacterial endocarditis

• congenital: bicuspid / unicuspid AoV is more vulnerable to degeneration and calcification

• Patients under 70: >50% have a congenital cause

• Patients over 70: 50% due to degenerative changes

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Aortic stenosis - pathophysiology

� CO

systemic hypotension � ventricular

pressure

� transmural

coronary artery

pressure

� coronary

blood flow

LVH

� cardiac O2

consumption

myocardial hypoxia (angina pectoris)

left heart failure

stim. of ventricular baroreceptors (?)

‘paradoxical’ vasodilation

�� BP

syncope

physical

exercise

vasodilation during exercise

arrhythmia

� ventricular filling

LA hypertrophy

atrial fibrillation

pulmonary hypertension

dyspnoe

Aortic stenosis - symptoms• Due to concentric LVH patients are many years asymptomatic with normal CO

• Angina pectoris:

• � coronary blood flow, � cardiac O2 consumption, coronary atherosclerosis

• Syncope:

• during exercise

• blood flows to dilated arteries in muscles � hypotension, cerebral hypoxia

• LV failure:

• severe AoS �� CO, blood congests in LV

• dyspnea

• Sudden cardiac death:

• late stage of AoS

• excercise � blood distributed to muscles �� myocardial hypoxia � ventricular ectopic regions � ventricular fibrillation

• ventricular fibrillation / severe cerebral hypoxia � sudden death

• � SBP, � difference between systolic and diastolic BP

• slow rising carotid pulse (pulsus tardus) and decreased pulse amplitude (pulsus parvus) = pulsus parvus et tardus

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Aortic stenosis - symptoms

Aortic stenosis – natural history

• The onset of symptoms is a poor prognostic indicator

• Average time to death after the onset of:

• angina pectoris: 3 years

• syncope: 3 years

• dyspnea: 2 years

• congestive heart failure: 1.5-2 years

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Aortic stenosis - auscultation• Systolic (ejection) click:

• sudden opening of AoV (due to �� ventricular-aortic pressure gradient)

• Systolic ejection murmur:

• “diamond” crescendo-decrescendo shaped, harsh

• to carotid aa., interscapular area

• sitting patient leaning forward

• peaks later as the severity of the stenosis increases

• loudness does NOT tell you anything about severity (in severe AoSt, murmur may � as CO falls)

• Soft and split S2:

• slow increase of pressure in aorta

• S4 gallop:

• due to LVH

• S3 sound:

• late, when LV dilates and fails


Aortic stenosis - evaluation• ECG:

• LVH, ST depression, T-wave inversion (LV ‘strain’) in leads I, aVL

• CXR:• poststenotic dilation of ascending aorta, aortic calcification

• ECHO:• most valuable test for diagnosis, quantification and follow-up of

patients with AoS

• LV size and function: LVH, dilation, and EF

• Doppler derived gradient and valve area (AVA)

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Aortic stenosis - treatment

• General:

• avoid dehydration, hypovolemia (protect from � CO)

• IE prophylaxis in dental procedures with a prosthetic AoV or history of endocarditis

• Medical:

• limited role since AS is a mechanical problem

• nitroglycerine – relieve angina pectoris

• vasodilators are relatively contraindicated in severe AoS

• Surgical:

• aortic balloon valvotomy: shows little benefit

• surgical replacement: definitive treatment

Aortic insufficiency (AoI)

• Mitral stenosis



• Aortic stenosis






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Aortic insufficiency

Etiology:

• Rheumatic fever: 2/3 of patients

• Atherosclerosis of AoV leaflets

• Bacterial endocarditis: • perforation or erosion of leaflets

• Syphilitic aortritis:• Scarred and retracted leaflets

• Bicuspid AoV with calcification

• Aortic root disease (aortic dilation):• Marfan syndrome

• Idiopathic dilation of the aorta

• Aneurysm of ascendent aorta

• Severe hypertension

Aortic insufficiency - pathophysiology

• during diastole blood flows at once from LA and aorta back to LV � volume overload of LV

• during systole blood volume from LV flows to the aorta increased by regurgitant volume � volume overload of aorta � dilation of aorta

• regurgitant volume (usually 20–80 ml, max. 200 ml/beat):

• opening area

• pressure difference during diastole

• duration of diastole

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Aortic insufficiency - pathophysiology

• Exercise:

tachycardia: shorter diastole �� regurgitation

peripheral muscle vasodilation �� mean diastolic pressure gradient

Aortic insufficiency - pathophysiologyRegurgitant volume

� diastolic BP � effective stroke volume

COMPENSATION� end-diastolic volume

� total stroke volume

effective SV normalized

years – decadesDECOMPENSATION

left heart failure

� effective SV

�� LV dilation

functional MiR � LV compliance

� left atrial pressure

Pulmonary edema

LV failure

ventricular dilation

Laplace’s law

� wall tension

LV hypertrophy

� O2 consumption

Myocardial hypoxia (angina pectoris)

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Aortic insufficiency - natural history

• Asymptomatic until 4th or 5th decade

• Rate of progression: 4-6% per year

• Progressive symptoms:

- Dyspnea: exertional, orthopnea, and paroxsymalnocturnal dyspnea

- Nocturnal angina: due to slowing of heart rate and reduction of diastolic blood pressure

- Palpitations: due to increased force of contraction

Aortic insufficiency - symptoms

• Acute severe AoI:• in infective endocarditis, aortic dissection, trauma• LV cannot dilate sufficiently to maintain stroke volume �� LV diastolic

pressure, LA pressure � pulmonary edema, cardiogenic shock

• Chronic severe AoI:• excentric LVH compensates volume overload for 10-30 years,

asymptomatic

• palpitations:• � systolic SV, pulsation of LV, ventricular extrasystoles

• peripheral vasodilation:• � SBP � vasodilation

• dizziness, sweating, bad tolerance to heat

• dyspnea

• weakness

• angina pectoris

• peripheral edemas

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Aortic insufficiency - symptoms• � pulse pressure:

• Quinke's sign: capillary pulsation (alternate flushing and paling) under the finger nails and finger skin, while pressure is applied to the tip of the nail

• Musset's sign: pulse-synchronous head nodding

• Muller's sign: uvula movement

• Taube's sign: booming “pistol-shot” sound over the femoral arteries

• � SBP (� SV), � DBP (regurgiting blood, peripheral vasodilation)

• pulsus celer et altus (Corrigan's pulse): rapidly rising pulse which collapses suddenly as arterial pressure falls rapidly

http://www.blaufuss.org/tutorial/index2.html

Aortic insufficiency - symptoms

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Aortic insufficiency - auscultation• Diastolic murmur:

• produced by the regurgitation

• high-pitched, blowing, decrescendo, heard over the base of the heart, aorta, left sternal border, radiates to Erb's point

• Mid-systolic ejection murmur:

• due to increased flow across the aortic valve

• heard at the base of the heart, transmitted along the carotid vessels

• Diastolic Flint-Austin murmur:

• soft, low-pitched, rumbling, mid-diastolic, over apex

• diastolic displacement of the anterior leaflet of the MiV by the AoI stream causing it to vibrate

• premature closing of MiV due to � intraventricular pressure

• crash of mitral and regurgiting flow

• Soft S1:

• Premature closing of MiV

• Absent S2:

• Incomplete closing of AoV

• S3 and systolic ejection sound


Aortic insufficiency - evaluation• ECG:

• LVH, ST depression, T-wave inversion in leads I, aVL, V5, V6 (LV strain)

• CXR:• enlarged cardiac silhouette and aortic root enlargement

• ECHO:• cornerstone for decision making and follow up evaluation• evaluation of the AoV and aortic root with measurements of LV

dimensions and function

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Aortic insufficiency - treatment

• Acute AoI:

• surgical emergency!

• positive inotrope: dopamine, dobutamine

• diuretics, vasodilators: nitroprusside

• avoid beta-blockers

• do not even consider a balloon pump

• General:

• IE prophylaxis in dental procedures with a prosthetic AV or history of endocarditis.

• Medical:

• Diuretics, vasodilators (ACEI, hydralazine)

• Surgical treatment:

• Definitive Tx

Tricuspid stenosis

• Mitral stenosis



• Aortic stenosis






rare

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Tricuspid stenosis

• normal TriV area: 7 cm2

• symptoms: TriV area < 1 cm2

Etiology:

• Rheumatic fever

• Associated with MiS

Pathophysiology:

• during diastole, less blood flows to RV, � CO

• � CO – normal / slightly � pressure in RV, pulmonary artery, LA despite the presence of MiS = masking MiS

• blood congests in RA, regurgitates from RA to jugular veins

Tricuspid stenosis

Symptoms:

• associated MiS: pulmonary congestion; if spontaneously improves = possible TriS

• atrial venous pulse, peripheral edemas, hepatomegaly, acsites

• � CO: fatigue

• atrial fibrillation

Auscultation: louder during inspiration

• Split S1

• diastolic murmur: heard along the left sternal margin, over the xiphoid process

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Tricuspid insufficiency

• Mitral stenosis



• Aortic stenosis






rare


Etiology:

• Rheumatic fever

• Congenital: Ebstein anomaly

• Functional:

• LV failure – pulmonary hypertension - dilation and insufficiency of RV

• Inferior MI

• Ischemic heart disease

• Dilated cardiomyopathy

Pathophysiology and symptoms:

• During systole, blood from RV regurgitates to RA � peripheral edemas, increased filling of jugular veins, hepatomegaly, ascites

• systolic pulsation of the liver, hepatocellular icterus, hemorrhage

• Dyspepsia: congestion in GIT

• Sudden relieve from dyspnea in LV failure = RV failure!!!

• Atrial fibrillation

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• Holosystolic murmur

• Carvallo`s sign:

• the systolic murmur found in TriI becomes louder during inspiration, it distinguishes it from MiI, which does not change with respiration

• S1: soft

• S3: new (adults), louder (children) - � diastolic filling

• S4: � atrial contraction

Pulmonary stenosis

• Mitral stenosis



• Aortic stenosis






rare

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Pulmonary stenosis

Etiology:

• Rare

• Rheumatic endocarditis

• Congenital

• Compensated by concentric RV hypertrophy

• � CO – fatigue, syncope


• Soft S2

• S4: new or louder

• Ejection click

• Systolic murmur

Pulmonary insufficiency

• Mitral stenosis



• Aortic stenosis






rare

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Pulmonary insufficiency

Etiology:

• Rare

• Functional: in advanced pulmonary hypertension

• Volume overload of RV


• Graham-Steell murmur:• regurgitant flow

• diastolic, decrescendo, blowing murmur

• Soft S2

Congenital Heart Disease(CHD)

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CHD: pathophysiology

• Anatomic and hemodynamic changes due to CHD may become significant in adulthood

• Pulmonary hypertension: Eisenmenger syndrome

• Erythrocytosis: in cyanotic CHD

• Infective endocarditis

Classification of CHDAcyanotic

Atrial septal defect (ASD)

Ventricular septal defect (VSD)

Patent ductus arteriosus (PDA)

Atrioventricular canal

Obstruction to blood

flow from ventricles

↑ pulmonary blood flow

(L → R)

Coarctation of aorta

Aortic stenosis

Pulmonic stenosis

CyanoBc (R → L)

↓ pulmonary blood flow

↑ / N blood flow

Tetralogy of Fallot

Tricuspid atresia

Ebstein’s Anomaly

Transposition of great arteries

Total anomalous pulmonary

venous return

Truncus arteriosus

Eisenmenger syndrome

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Classification of CHDAcyanotic








(L → R)


Aortic stenosis

Pulmonic stenosis

CyanoBc (R → L)


↑ / N blood flow

Tetralogy of Fallot

Tricuspid atresia

Ebstein’s Anomaly



venous return

Truncus arteriosus


Coarctation of the aorta

• Narrowing or constriction of the aorta distal to the origin of the left subclavian artery near the insertion of the ligamentum arteriosum

• Systolic BP higher in upper extremities (headache, epistaxis)than in lower extremities (cold skin, claudication); diastolic BP are similar

• Absent or weak femoral pulses

• Harsh systolic murmur heard in the back

Illustration taken from http://www.chd-diagrams.com1. Coarctation of the aorta

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Valvular aortic stenosis• Bicuspid aortic valve

• very common type, usually asymptomatic in children

• Thickened, fibrotic, calcifiedmalformed aortic leaflets

• Left ventricular hypertrophy

• Harsh systolic murmur, systolic ejection click

Illustration taken from http://www.chd-diagrams.com

1. Bicuspid aortic valve with stenosis

2. Left ventricular ypertrophy

Pulmonary stenosis

• No symptoms in mild or moderately severe lesions

• Cyanosis and right-sided heart failure in patients with severe lesions

• High pitched systolic ejection murmur maximal in second left interspace

• Ejection click often present

Illustration taken from http://www.chd-diagrams.com1. Pulmonary valve stenosis

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Classification of CHD

Acyanotic








(L → R)


Aortic stenosis

Pulmonic stenosis

CyanoBc (R → L)


↑ / N blood flow

Tetralogy of Fallot

Tricuspid atresia

Ebstein’s Anomaly



venous return

Truncus arteriosus


Atrial septal defect

• Acyanotic; asymptomatic, or dyspnea on exertion


• Pulmonary hypertension

• Conversion to right-to-left shunt –cyanosis

• Atrial fibrillation

• Fixed, widely split second heart sound


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Ventricular septal defect

• Asymptomatic if defect is small

• Many of the defects will close spontaneously by age 7-8 y.

• Heart failure with dyspnea, frequent respiratory infections


• Pulmonary hypertension

• Conversion to right-to-left shunt –cyanosis

• Pansystolic murmur maximal at the left sternal border


1. Perimembranous ventricular septal defect

Patent ductus arteriosus• Poor feeding, respiratory distress, and

frequent respiratory infections in infants with heart failure

• Murmur usually systolic, sometimes continuous, “machinery”

• Indomethacin, a prostaglandin E1

inhibitor may close a PDA


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Complete atrioventricular canal

1. Inlet ventricular septal defect

2. Ostium primum defect (ASD Type I)

3. Left AV-Valve cleft

4. Right AV-Valve cleft Illustration taken from http://www.chd-diagrams.com

• Deficiencies of both atrial and ventricular septal cushions and

abnormalities of both mitral and tricuspid valves

Complete atrioventricular canal• Heart failure common in infancy

• Partial and complete AV canal defects frequently accompany Down’s syndrome

• Cardiomegaly, blowing pansystolic murmur, other variable murmurs

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Acyanotic








(L → R)


Aortic stenosis

Pulmonic stenosis

CyanoBc (R → L)


↑ / N blood flow

Tetralogy of Fallot

Tricuspid atresia

Ebstein’s Anomaly



venous return

Truncus arteriosus


Transposition of the great arteries• Aorta from right ventricle,

pulmonary artery from left ventricle

• Cyanosis from birth, hypoxic spells sometimes present

• Heart failure often present

• Cardiac enlargement and diminished pulmonary artery segment on x-ray

• Anatomic communication must exist between pulmonary and systemic circulation, VSD, ASD, or PDA

1. D-Transposition of the great arteries

2. Persistent ductus arteriosus

3. Patent foramen ovale Illustration taken from http://www.chd-diagrams.com

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Total anomalous pulmonary venous connection (TAPVC)

• Pulmonary veins do not make a direct connection with the left atrium.

• Supracardiac TAPVC

• Cardiac TAPVC

• Infracardiac TAPVC

• Blood reaches the left atrium only through an atrial septal defect or patent foramen ovale.

TAPVC: supracardiac

to the innominate vein to the superior vena cava

1. Pulmonary veins

2. Persistent left superior caval vein3. Inomminate vein

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TAPVC: cardiac

to the coronary sinus to the right atrium

1. Pulmonary veins 2. Coronary sinus

TAPVC: infracardiac

infradiaphragmatically to the portal circulation

1. Inferior caval vein 2. Pulmonary vein

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TAPVC: symptoms

• No obstruction to pulmonary venous return:

• Supracardiac, cardiac type

• Mild cyanosis

• subtle symptoms of heart failure

• Obstruction to pulmonary venous return:

• Infracardiac type

• severe neonatal cyanosis, pulmonary edema, pulmonary hypertension

Truncus arteriosus

• Single large vessel overrides the ventricular septum and distributes all the blood ejected from the heart

• Large VSD is present

• Common in DiGeorge syndrome


1. Truncus communis arteriosus

2. Truncus valve

3. Ventricular septal defect

4. Pulmonary arteries

5. Right ventricular hypertrophy

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Acyanotic








(L → R)


Aortic stenosis

Pulmonic stenosis

CyanoBc (R → L)


↑ / N blood flow

Tetralogy of Fallot

Tricuspid atresia

Ebstein’s Anomaly



venous return

Truncus arteriosus


Tetralogy of Fallot


2. Valvular pulmonic stenosis

3. Overriding aorta

4. Right ventricular hypertrophy due to

shunting of blood

• cyanosis, clubbing, dyspnea with

feeding, poor growth, hypercyanotic

"tet" spells, squatting


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Tricuspid atresia• Absence of the tricuspid valve

• Blood flows from right atrium to left atrium through foramen ovaleor atrial septal defect

• Early cyanosis


1. Tricuspid atresia

2. Atrial septal defect

3. Hypoplastic right ventricle


5. Pulmonary valve stenosis

Ebstein’s anomaly

• Septal and posterior leaflets of the tricuspid valve are small and deformed, usually displaced toward the right ventricular apex

• Most patients have an associated ASD or patent foramen

• Cyanosis and arrhythmias in infancy are common

1. Apical displacement of the septal leaflet

2. Tethering of the anterior leaflet

3. Tricuspid regurgitation

4. Atrial septal defect Illustration taken from http://www.chd-diagrams.com

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Kristína Repová, M.D., PhD.

[email protected]

Institute of Pathophysiology, Faculty of Medicine, Bratislava

Heart valve disease, congenital heart disease · mitral regurgitation, anemia, hyperthyroidism,...

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Transcript of Heart valve disease, congenital heart disease · mitral regurgitation, anemia, hyperthyroidism,...