Miscellaneous Echo finding (brief notes)

Ehlers-Danlos Syndrome:

 A heterogeneous group of connective tissue disorders with simple Mendelian inheritance. The cardinal manifestations of EDS are hyperextensible (stretchy) skin, hypermobile joints and easy bruising.

Echo/Doppler: Aortic root dilation, sinus de Valsalva aneurysm, Dilation of the proximal innominate artery, MVP, AVP, VSD, ASD, Tetralogy of Fallot, Aortic coarctation, Bicuspid AV, and Dextrocardia.

Fabry’s Disease:

          An X-linked recessive inborn error of glycosphingolipid metabolism.

          Echo/Doppler: MVP, increased LV wall thickness/mass, Aortic root dilation, MR, diastolic dysfunction.

Friedreich’s Ataxia:

          Cardiac abnormalities are a characteristic feature of the autosomal recessively inherited spinocerebellar degeneration known as FA, which is the most common hereditary ataxia.

          Echo/Doppler: Concentric Hypertrophy, Globally decreased LV systolic function, EF and FS. Increased left Atrial dimension.

Glycogen storage disease:

          A group of inheritable disorders of glycogen metabolism, resulting from specific enzymatic defects; Pompe’s disease (GSD type IIa) is a classic form of infantile GSD and is autosomal recessive.

          Echo/Doppler: severe thickness of the IVS, free wall and LVPW with a tumor-like appearance of the papillary muscles with small LV cavity. Poor global LV systolic function.

Holt-Oram (Heart-Hand) Syndrome:

          An autosomal dominant syndrome characterized by skeletal abnormalities and congenital cardiac defects.

          Echo/Doppler: ASD (ostium secundum), dilation of the right heart and paradoxical motion of IVS consistent with RVOL. VSD (trabecular type), may present with complex congenital heart disease/vascular malformations, Nonobstructive CM and MVP.

Loeys-Dietz syndrome (Aortic Aneurysm Syndrome):

          Autosomal dominant aortic aneurysm syndrome characterized by the triad of arterial tortuosity and aneurysms, hypertolerism and cleft palate. Aggressive arterial aneurysm (mean age of death 26 years of age), vascular disease may be widespread, and increased pregnancy related complications.

Marfan’s Syndrome:

          A hereditary condition of connective tissue, bones, muscles, ligaments and skeletal structures.  

          Echo/Doppler: Proximal aortic dilatation, multivalvular prolapse, dilated mitral annulus, MAC, left Atrial compression, LVVOL, AI, MR, aortic dissection (most common cause of death) and CAD.

Lyme disease:

          Caused by tick-borne spirochete, Borrelia burgdorferi, initial infection marked by rash, followed by weeks to months by involvement of other organ systems, including the heart, neurologic system and joints.

          Echo/Doppler: severe AV node block associated with syncope; DCM.

Noonan’s Syndrome:

          A inherited autosomal dominant manner and is characterized by congenital heart disease, short stature, abnormal facies and somatic features of Turner’s Syndrome but normal karyotype.

          Echo/Doppler: PV stenosis, Secundum ASD, VSD, PDA, and localized anterior septal hypertrophy.

Phen-fen:

          The appetite suppressants phentermine, fenfluramine and dexfenfluramine, alone or in combination, have been implicated as causing valvular disease; complication uncommon with less than 6 months of use.

          Echo/Doppler: thickened MV/Chordae Tendineae, thickened AV, mild or greater AR, mod or greater MR, pulmonary HTN.

 

Bibliography

Armstrong, W. F., & Ryan, T. (2010). Feigenbaum's Echocardiography. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins.

Reynolds, T. (2013). The Echocardiographer's Pocket Reference. USA: Arizona Heart Foundation.

PREDICTION OF INTRACARDIAC PRESSURES (Reynolds, 2013)

2-D echocardiography combined with cardiac Doppler may be utilized to predict intracardiac pressures.

Right Atrial Pressure (RAP)

v Normal 2 to 5 mmHg, to approximate the RAP, examine the IVC by 2-D and substitute one of the following values for the actual RAP.

Variable	Normal (0-5(3)mmHg)	Intermediate (5-10(8) mmHg)		High (15 mmHg)
IVC diameter	Less than 2.1 cm	Less than 2.1 cm	More than 2.1 cm	More than 2.1 cm
Collapse with sniff	More than 50 %	Less than 50 %	More than 50 %	Less than 50 %

 

Secondary indices of elevated RAP:

ü Restrictive filling

ü Tricuspid E/E’ more than 6

ü Diastolic flow predominance in hepatic veins (systolic filling fraction less than 55 %).

 

v Dilated hepatic veins with a dilated inferior vena cava suggest increased right Atrial pressure.

 

v A dilated coronary sinus (normal 4 to 8 mm as measured in the A4C with a posterior tilt) suggests increased RAP; it may be dilated also with persistent left superior vena cava, coronary artery AV fistula, anomalous hepatic vinous drainage to the left coronary sinus, and total anomalous pulmonary venous return or severe tricuspid regurgitation.

 

 

 

 

 

 

 

 

 

 

 

 

v In the absence of significant tricuspid regurgitation (TR), a right atrium that is increased in dimension especially when compared to the left atrium, suggests increased RAP.

 

v A right atrium maximal volume measured at end-systole of more than 45 +/- 14 cm3 suggests a mean RAP of more than 8 mmHg.

v A right Atrial maximal volume measured at end-diastole of more than 30 +/- 15 cm3 suggests a mean RAP of more than 8 mmHg.

v Interatrial septal deviation towards the left atrium may indicate increased RAP.

v A PW Doppler tricuspid valve E/A ratio of equal or more than 1.1 may indicate a mean RAP of > 8 mmHg, assumes the absence of right ventricular inflow tract obstruction.

 

v A dagger shaped CW tricuspid regurgitation velocity spectral display suggests increased RAP.

 

 

v A decreased right ventricular IVRT (normal 54 +/- 3.55 msec) suggests increased RAP.

 

 

 

v A patient with left heart disease and pulmonary hypertension may eventually develop elevated right heart pressures.

 

Bibliography

Reynolds, T. (2013). The Echocardiographer's Pocket Reference. USA: Arizona Heart Foundation.

PERICARDIAL DISEASE (my class notes)

The pericardium is made up of two layers. The epicardium (visceral pericardium) is a serous membrane which lies directly adherent to the heart and the outer layer is called the fibrous (parietal) pericardium. The normal thickness of the parietal pericardium is approximately 2 mm. there is approximately 10 to 50 mL of fluid normally between the epicardium and the fibrous pericardium. This normal pericardial fluid may be seen as an echo-free space during ventricular systole. When the echo-free space persists throughout the cardiac cycle, a pericardial effusion is considered present. When quantitating the amount of pericardial fluid by echocardiography the fluid should be measure during ventricular diastole. Intrapericardial stands or fibrin strands within pericardial effusion most likely indicate inflammation or long-standing pericardial effusion.

The most common presenting symptom of acute Pericarditis is Chest pain; other symptoms include dyspnea, fever and cough. The chest pain associated with acute Pericarditis may be relieved by the patient sitting up and leaning forward.

The expected echocardiographic finding for Pericarditis is a pericardial effusion but patients may have Pericarditis without pericardial effusion (dry Pericarditis). Additionally pericardial effusion can occur in the absence of pericardial inflammation such as in post-operative cardiac surgery, chronic renal disease, collagen vascular disease, cardiac trauma, malignancy, AIDS and hypothyroidism.

The best guideline for differentiating pericardial effusion from pleural effusion by two dimensional echocardiography is: pericardial effusion is located anterior to the descending aorta; pleural effusion is present posterior to the descending aorta; in addition, a large pericardial effusion usually surrounds the heart.

Pulsus paradoxus is present when there is an exaggerated inspiratory decline in arterial blood pressure of more than 10 mmHg. Pulsus paradoxus is associated with cardiac tamponade for two reasons: filling of both ventricles against a common stiffness and respiratory changes in the venous pressure differential (systemic versus pulmonary) alternately favoring right and left ventricular filling. Respiratory variations of the cardiac Doppler signal of the atrioventricular valves suggest pulsus paradoxus.

In cardiac tamponade the right ventricular diastolic collapse occurs in early diastolic when the right ventricular volume and pressure are at lowest levels. Right ventricular diastolic collapse can be observed in the parasternal long axis view, parasternal short axis view, apical four chambers and the subcostal four chamber view.

With a large anterior and posterior pericardial effusion the heart may move freely within the pericardial cavity. This type of motion called the swinging heart syndrome tends to occur in large pericardial effusions. The swinging may cause the phenomenon of electrical alternant.

Marked (more than 25%) respiratory variation of tricuspid valve (increase in inspiration; decrease with expiration) and mitral valve (decrease in inspiration; increase with expiration) peak flow velocities and/or velocity time integrals is a strong indicator for the presence of cardiac tamponade. The orders in which changes occur are fairly predictable. Changes in the tricuspid occur first, followed by changes in the mitral valve with right atrial collapse being the next change and right ventricular diastolic collapse occurring last. Hepatic vein flow in cardiac tamponade mimics the flow changes of the tricuspid valve. The hepatic vein will have a reduction or reversal of diastolic flow expiration.

Constrictive Pericarditis results in the impairment of diastolic filling of the heart which may result in diastolic heart failure. Diastolic filling is impaired because of the constraint the pericardium places on the heart. Intracardiac filling pressures are usually increased in patients with constrictive Pericarditis. Constrictive Pericarditis should be a differential diagnosis in patients who present with congestive heart failure and normal global ventricular systolic function. Virtually all filling of the ventricle occurs in very early diastole. This abnormal pattern of diastolic filling is reflected in the characteristic dip-and-plateau (square root sign) waveforms in both the right and the left ventricles. The rapid rise in pressure after the early diastolic correspond to the period of rapid diastolic filling while the plateau phase correspond to the period of mid and late diastole when there is little additional expansion of ventricular volume. This can be expressed by the inflow patterns of the mitral valve and tricuspid valve with increased E/A ratio (more than 1.5) and shortened deceleration time ( less than 140 msec).

Causes of Constrictive Pericarditis include prior pericardiotomy, idiopathic, Pericarditis, radiation, infection and collagen vascular disease. Tuberculosis was at one time considered the leading cause of constrictive Pericarditis but idiopathic and prior pericardiotomy are the most common reason for Constrictive Pericarditis currently.

A thickened pericardium more than 2 mm, interventricular/inter-Atrial septal bounce (shudder), bound-down appearance of the ventricular walls with lack of pericardial slide, inferior vena cava plethora, normal Atrial dimensions, normal ventricular dimensions  and normal global ventricular systolic function are the echocardiographic findings associated with constrictive Pericarditis.

Pulsed wave Doppler in Constrictive Pericarditis:

Ø Mitral flow: restrictive pattern (increase E/A ratio, shortened DT)

Ø Increased peak velocity across the mitral valve with expiration

Ø Hepatic flow vein: expiratory flow decrease or reversal

Ø Tissue Doppler in mitral valve annulus is Normal E’ wave peak velocity (more or equal to 8 cm/s) and the E/E’ ratio is normal (more than 8), it is called annulus paradoxus

FARMACOTERAPIA EN LA INSUFICIENCIA CARDIACA (I):

El objetivo de la Farmacoterapia en el fallo cardiaco es reducir la morbilidad y prevenir las complicaciones. Dentro de los medicamentos que ayudan a aliviar los síntomas, junto al uso del oxigeno, se encuentran los siguientes (Dumitru, 2014):

·         Diuréticos: reducen el edema mediante la reducción  del volumen sanguíneo y la presión venosa.

·         Vasodilatadores: reducen la pre y post-carga.

·         Digoxina: puede causar un leve incremento del gasto cardiaco.

·         Agentes Inotrópicos: ayudan a restaurar la perfusión del órgano y reducir la congestión.

·         Anticoagulantes: reducen el riesgo de tromboembolismo.

·         Beta-Bloqueadores: por modificación neurohormonal,  mejoran la fracción de eyección del ventrículo izquierdo (LVEF), previenen arritmias, y controlan la frecuencia ventricular.

·         IECA: por modificación neurohormonal, vasodilatación y mejoramiento de la LVEF.

·         Bloqueadores de los receptores de la angiotensina II: también por modificación neurohormonal, vasodilatación y mejoramiento de la LVEF.

·         Analgésicos: manejo del dolor.

Medicamentos que pueden exacerbar la insuficiencia cardiaca y que deben ser evitados:

·         Anti-inflamatorios no esteroideos: pueden causar retención de sodio y vasoconstricción periférica, además pueden atenuar la eficacia y elevar la toxicidad de diuréticos y IECAs.

·         Bloqueadores de los canales de calcio: pueden empeorar el fallo cardiaco e incrementar el riesgo de eventos cardiovasculares, solo los bloqueadores de los canales de calcio vasoselectivos han demostrado no tener efectos negativos en la supervivencia.

·         Drogas anti-arrítmicas excepto las de Clase III: pueden tener efectos cardiodepresivos y facilitar las arritmias, solo la amiodarona  y el dofetilide han demostrado no tener efectos negativos en la supervivencia

 Bibliography

Dumitru, I. (2014, Apr 23). medscape.com. Retrieved from emedicine.medscape.com: http://emedicine.medscape.com/article/163062-medication

 

INFECTIVE ENDOCARDITIS: (my class notes)

Fever is the most common symptom (80 to 85%) of infective endocarditis. Additional symptoms include chills, sweats, anorexia, weight loss, malaise, dyspnea, cough, stroke, headache, nausea/vomiting, myalgia/arthralgia, abdominal pain, back pain and confusion.  The onset of symptoms is estimated to be less than two weeks in patients with native valve endocarditis. The perioperative period post-cardiac valve replacement is two to five months or longer. Heart murmurs are noted in 80 to 85% of patients with infective endocarditis. The classic triad of infective endocarditis is fever, new murmur and positive blood cultures. The classic echocardiographic findings in infective endocarditis are valvular vegetation and valvular regurgitation.

Complications of infective endocarditis include:

Ø Congestive heart failure

Ø Embolization

Ø Valve ring abscess

Additional complications include valve leaflet vegetation, disruption (e.g. flail) with resultant regurgitation, perforation, aneurysm, fistula, dehiscence of prosthetic valve, pericardial effusion and hemodynamic compromise (e.g. valvular regurgitation, premature mitral valve closure, restrictive mitral valve inflow pattern, valvular stenosis (rare) and shunt).

Infective endocarditis is a greater risk in patients with prosthetic heart valve; the classic clinical setting for infective endocarditis is pre-existent valvular heart disease (e.g. rheumatic, myxomatous, congenital, prosthetic heart valve, intravenous drug abuse). A mode of infection (e.g. dental, surgical, and traumatic) is often identifiable. If a patient is an intravenous drug abuser normal right sided cardiac valves can be affected.

Vegetations are common to all types of infective endocarditis. They are situated most frequently on the valvular leaflets and less often on the endocardium of the ventricles or of the left atrium (McCallum’s patch of rheumatic carditis) and on the pulmonary or other arteries. The expected cardiac Doppler finding for infective endocarditis is valvular regurgitation. Approximately 15 % of patients do not have a new murmur due to valvular regurgitation with one possible explanation being that the vegetation is located on the base of the leaflet which may not disrupt valve closure. Valvular stenosis is a rare complication of native valve infective endocarditis.

The usual site of attachment for vegetation is on the atrial side (low pressure side) of the mitral and tricuspid valve leaflets. Aortic and pulmonary valve vegetations are usually found on the ventricular (low pressure side) of the valve. When the diameter of the vegetation exceeds 10 mm 50 % of patients develop at least one complication of infective endocarditis. In tricuspid valve endocarditis, pulmonary embolism is the most common complication.

Valve ring abscess is an uncommon but a serious typical complication of infective endocarditis and usually involves the aortic valve ring. Rupture of aortic ring abscess can occur creating a fistula.

Valve ring abscess usually presents as an area of echolucency (if cystic) or echoreflectant (if solid) around the valve ring or myocardium. Abscesses may be found in the aortic posterior annulus, peri-annular area, aortic-mitral intervalvular fibrosa, posterior aortic root and interventricular septum. For prosthetic heart valves, abscesses are usually seen around the sewing ring.

The sensitivity of detecting a vegetation with transthoracic two-dimensional echocardiography (TTE) is 65 to 80 % and with transesophageal echocardiography (TEE) it is95 %. It has been proposed that all patients with suspected infective endocarditis should undergo TEE examination. The clinical utility of cardiac magnetic resonance imaging has not been determined but has been useful in determining the presence of perivalvular extension of infection, aortic root aneurysm and fistulas.

 

PULMONARY REGURGITATION (my class notes)

     By far the most common cause of pathologic pulmonary regurgitation is dilation of the valve ring secondary to pulmonary hypertension. The second most common cause is infective endocarditis. Iatrogenic significant pulmonary regurgitation may be present in patients following treatment for pulmonary valve stenosis or tetralogy of Fallot.

     Isolated pulmonary regurgitation may lead to right ventricular volume overload. Right ventricular volume overload (RVVO) is diagnosed by the echocardiographic findings of right ventricular dilatation and paradoxical interventricular septal motion. RVVO may be diagnosed with two-dimensional echo as right ventricular dilatation and flattening of the interventricular septum during diastole with restoration of the normal circular configuration during ventricular systole best seen in the parasternal short-axis of the left ventricle at the level of the papillary muscles.

     Color Doppler findings indicate significant pulmonary regurgitation:

Ø Wide jet width at origin

Ø Jet width/Right ventricular outflow tract width more than 70 %

Ø Holodiastolic flow reversal in the main pulmonary artery

Ø Jet length more than 10 mm

     Pulmonary artery end-diastolic pressure can be calculated by the formula:

     PAEDP (mmHg) = 4xend-diastolic velocity PR2 + RAP (mmHg)

     Normal PAEDP is 4 to 12 mmHg

     The estimation of the PAEDP reflects the pulmonary artery wedge pressure.

     Mean Pulmonary Artery Pressure may be calculated by the formula:

     MPAP (mmHg) = 4x peak velocity PR2

     The normal range for the MPAP is 9 to 18 mmHg.

 

MITRAL VALVE STENOSIS (my class notes)

Classic M-mode findings for Mitral Stenosis:

v Thickened mitral valve leaflets

v Decreased mitral valve E-F slope

v Anterior motion of the posterior mitral valve leaflet

 

 

 

 

 

 

Two dimensional echo finding for rheumatic MV stenosis:

v Hockey-stick appearance of the anterior mitral valve leaflet

v Increase left Atrial dimension

v Thickened MV leaflets and subvalvular apparatus

Other Two dimensional echo/Doppler finding include:

v D-shaped left ventricle

v Left Atrial thrombus

v Abnormal interventricular septal wall motion

v Increase right heart dimension

v Increased TV regurgitation jet velocity

v Increased E velocity

v Increased Pressure half-time

v Turbulent flow

MVA:  Severe stenosis = less than 1.0 cm2

            Moderate           = 1.1 to 1.5 cm2

            Mild stenosis     = 1.6 to 2.5 cm2

Normal MVA = 4 to 6 cm2

MVA (cm2) = 220/PHT (msec)

The abnormal range for PHT in patient with MV stenosis is 90 to 400 msec. MV stenosis is considered severe when PHT is 220 msec or longer.

PHT (msec) = Deceleration Time x 0.29

Mean Pressure gradient (tracing CW Doppler spectral wave form)

v Severe stenosis = more than 10 mmHg

v Moderate           = 4 to 10 mmHg

v Mild stenosis     = less than 4 mmHg

SEVERE MITRAL VALVE STENOSIS:

Ø MEAN PRESSURE GRADIENT MORE THAN 10 mmHg

Ø MITRAL VALVE AREA LESSN THAN 1.0 cm2

Ø PRESSURE HALF TIME MORE THAN 220 msec