Cardiomyopathies (Non-Ischemic), Hypertensive and Pulmonary Heart Disease Susan A. Raaymakers, MPAS,PA-C, RDCS (AE)(PE) Radiologic and Imaging Sciences - Echocardiography Grand Valley State University, Grand Rapids, Michigan [email protected] Basic Review of Heart Walls Three Heart Walls Epicardium Myocardium Endocardium Basic Review of Heart Walls Three Heart Walls Epicardium Myocardium Endocardium Basic Review of Heart Walls Three Heart Walls Epicardium Myocardium Endocardium Basic Review of Heart Walls Epicardium Includes: Nerves to heart and coronary blood vessels Connective tissues Basic Review of Heart Walls Myocardium Muscular layer composed of bands of cardiac fibers Basic Review of Heart Walls Endocardium Contains branches for electrical conduction system Pathway for blood supply to the valves Definition of Cardiomyopathy Primary disease of the myocardium Three Basic Types of Cardiomyopathies Dilated Hypertrophic Restrictive Overlap particularly between dilated and restrictive Dilated Cardiomyopathy (DCM) Dilated Cardiomyopathy Four-chamber enlargement with impaired systolic function of both ventricles Physiology is characterized by: Impaired left ventricular contractility Reduced cardiac output Elevated left ventricular end-diastolic pressure Dilated Cardiomyopathy Potential Causes Idiopathic Toxins Alcohol Medications Cobalt Snake bites Metabolic Thiamine deficiency Acromegaly Sickle cell anemia Non-dynamic Dilated Cardiomyopathy Potential Causes-continued Peripartum Non-compaction Infectious Systemic diseases e.g. Chagas’ disease Immune-mediated injury Inherited disorders Duchenne’s muscular dystrophy Sick cell anemia Dilated Cardiomyopathy Non-Compaction (“spongy myocardium”) Congenital cardiomyopathy of children and adults resulting from arrested myocardial development during embryogenesis. Circulation May 11, 1999 vol. 99 no. 18 2475 Dilated Cardiomyopathy Non-Compaction Prior to formation of the epicardial coronary circulation (about 8 weeks of life) The myocardium Increased surface area permits Meshwork of interwoven myocardial fibers that form trabeculae and deep trabecular recesses. Perfusion of the myocardium by direct communication with the left ventricular cavity. Normally, as the myocardium undergoes gradual compaction, the epicardial coronary vessels form. . Dilated Cardiomyopathy Non-Compaction (“spongy myocardium”) Echocardiographic results A thin epicardium with extremely hypertrophied endocardium and prominent trabeculations with deep recesses. Often apically localized Compaction would normally proceed from base to apex, and from epicardium to endocardium. http://www.youtube.com/watch?v=vWnlSIonta0&feature =related Echocardiographic Approach Non-Compaction Cardiomyopathy (CM) Left ventricular systolic function Qualitative global and regional function Qualitative end-diastolic and end-systolic dimensions or volumes Ejection fraction Right ventricular systolic function Qualitative size and systolic function Pulmonary artery systolic pressure Dilated Cardiomyopathy Chagas Disease (American trypanosomiasis) Parasitic infection Rare in North America and Europe (Endemic in South and Central America) Early acute symptoms: mild local swelling at site of swelling Serious chronic symptoms as many as 20 years later: heart disease and malformation of intestines Chagas Disease LV apical aneurysm with minimal involvement of the ventricular septum Apical obliteration with a small ventricular chamber Atrial enlargement Atrioventricular valve regurgitation Non-dynamic image Circulation. 2007; 115: 1124-1131 Echocardiographic Approach Clinical Utility Echocardiography considerations Systolic and diastolic function Chamber sizes Associated valvular disease Pulmonary artery pressures Periodic echocardiograms essential for optimal care for medications and for need of cardiac transplantation Cardiac catheterization is indicated for pulmonary vascular resistance in heart transplant candidates Echocardiographic Approach to Dilated Cardiomypathies Increased E-Point Septal Separation (EPSS) “B-Bump” (AC shoulder) Left ventricular dilation & reduced mitral leaflet motion D/T low transmitral flow rates Delayed mitral valve closure Decreased aortic root motion with early closure of the aortic valve Reduced left atrial filling and emptying Echocardiographic Approach to Dilated Cardiomypathies Doppler Reduced aortic ejection velocity Reduced stroke volume, Compensatory mechanisms (including left ventricular dilation) result in normal stroke volume at rest in many individuals Associated mitral regurgitation Often moderate in severity Due to ventricular dilation and abnormal alignment of the papillary muscles Echocardiographic Approach to Dilated Cardiomyopathies Doppler Reduced dP/dt Slow rate of rise in velocity of the MR jet Reduced rate of increase in left ventricular pressure in early systole (dP/dt) Diastolic Dysfunction Early in disease course Grade I E<A Echocardiographic Approach Limitations/Technical Considerations Usually cannot establish the etiology of dilated cardiomyopathies Fairly uniform regardless of etiology of disease Exception: Chagas’ Heart Disease Dilated Cardiomyopathy Large LA and LV, All Walls are Equally Hypokinetic 17-01A Feigenbaum Dilated Cardiomyopathy Same Patient 17-01B Feigenbaum Tricuspid Regurgitation Due To Dilated Cardiomyopathy 12-030a Feigenbaum Tricuspid Regurgitation Due To Dilated Cardiomyopathy – Same Patient 12-030b Feigenbaum Dilated CM with Preserved BulletShaped Geometry (Long Axis Significantly > Short Axis) 17-02 Feigenbaum Dilated CM without Preserved BulletShaped Geometry (Long Axis Not Significantly > Short Axis) 17-03 Feigenbaum Dilated Cardiomyopathy With Apical Thrombi 17-21 Feigenbaum Mobile Thrombus Connected to Laminar Thrombus 17-22 Feigenbaum Papillary Muscle Displacement Due To Dilated Cardiomyopathy 17-23 Feigenbaum Papillary Muscle Displacement Due To Dilated Cardiomyopathy 17-24 Feigenbaum Hypertrophic Cardiomyopathy (HCM) 2011 Hypertrophic Cardiomyopathy Association Definition of HCM HCM is characterized by left ventricular (LV) hypertrophy without dilatation in the absence of another cardiac or systemic condition that could be responsible for the extent of hypertrophy that is present. Mutations involving the gene encoding proteins in the sarcomere are responsible for the majority of genetically mediated cases. https://www.4hcm.org/2011_accf_aha_guidelines.html Basic Principles Autosomal dominant inherited disease of the myocardium Related to abnormalities in the ß-myosin heavychain gene HCM Predominant Features Asymmetric hypertrophy of the LV Normal ventricular systolic function Impaired diastolic LV function Subaortic dynamic obstruction in some individuals Non-dynamic Other Important Clinical Features High risk of sudden death (especially during exertion) Symptoms of angina Syncope Systolic murmur on auscultation Pattern of Left Ventricular Hypertrophy Quite variable “Classic” septal hypertrophy: Anterior segment of the ventricular septum (Type I) Anterior and posterior segments of the septum (Type II) with sparing of the lateral, posterior and inferior walls Extensive thickening with normal wall thickness seen only in basal segment of the posterior wall (Type III) Isolated apical hypertrophy (Type IV) Echocardiographic Approach Multiple tomographic views Attention in parasternal long axis view to inferolateral/inferoseptal basal wall between papillary muscle and mitral annulus Wall is in this region is not thickened in patients with HCM Non-dynamic Left Ventricular Diastolic Pressure Impaired diastolic relaxation and elevation of left ventricular end-diastolic pressure Increased duration & velocity of the pulmonary vein a-reversal Prolonged IVRT Reduced E velocity Enhanced A velocity Subaortic Obstruction -Dynamic Outflow Tract Obstruction Apposition of the anterior leaflet of the mitral valve against the hypertrophied ventricular septum in systole Subaortic Obstruction - Dynamic Outflow Tract Obstruction Dynamic rather than fixed Occurs only in mid to late systole Maximum gradient occurs in late systole Subaortic Obstruction -Dynamic Outflow Tract Obstruction Systolic anterior motion of the mitral leaflet Mid-systolic closure of the aortic valve Late peaking high velocity flow in the outflow tract Variability in the severity of the obstruction with certain maneuvers Aortic leaflets may be sclerotic because of the long-term effect of a turbulent jet http://www.youtube.com/watch?v=Y7JUVTXHBs0&feature=endscreen& NR=1 Subaortic Obstruction -Dynamic Outflow Tract Obstruction Post PVC or PAC (increases contractility) Valsalva maneuver (decreased preload) Stress Echocardiography with CW before and after exercise Inhalation of amyl nitrate Brief decrease in preload (venodilation) Decrease in afterload (arterial dilation) Subaortic Obstruction -Dynamic Outflow Tract Obstruction Inhalation of amyl nitrate (decreased afterload and preload Direct Physician supervision CW Doppler before, during and after administration while maintain parallel intercept angle Moderate degree of tachycardia may occur but duration of action of medication is brief Physician monitors HR, rhythm, symptoms, and BP http://www.justoneshot.org.uk/drug_amyl.htm Dynamic Outflow Tract Obstruction M-Mode “Contact lesion” on the ventricular septum Midsytolic closure of the aortic valve followed by course fluttering of the leaflets Left: SAM (arrows) Right: Mid-systolic closure of the aortic valve (arrow) following by coarse fluttering of the leaflets Systolic Anterior Motion of the Mitral Valve (SAM) Dynamic Outflow Tract Obstruction Doppler Provide more direct evaluation than imaging techniques of: Presence, Location, and Degree of dynamic obstruction Dynamic Outflow Tract Obstruction Doppler Apical Views Using PW Doppler sample volume Proximal to outflow obstruction Slowly moved from apex toward the base recording the velocity at each step Velocity is normal Site of obstruction Velocity increases abruptly reflecting the degree of the obstruction (Bernoulli Equation 4V2) Dynamic LVOT Apical Views CW Doppler Shows a late-peaking high velocity systolic jet in patient with dynamic outflow tract obstruction Remember CW measures velocities along entire length of the ultrasound beam The obstruction may be due apex rather than SAM Need PW to rule this out Systolic Motion of the Anterior Mitral Leaflet (SAM) Note the relatively narrow width of the turbulent jet at the level of the mitral valve (arrows). 17-54 Feigenbaum Mitral Valve Abnormalities MR results from SAM leading to: Late systolic failure of coaptation Posteriorly directed jet Autopsy & echo studies: Increased surface area & longer length (particularly the anterior leaflet) Degree of coaptation: excessive Posteriorly displaced coaptation plane 10%: anomalous papillary muscle anatomy with direct insertion of the papillary muscle into the leaflet Anaesthesia Volume 63, Issue 10, Limitations/Technical Considerations HCM can be mimicked Chronic hypertension (especially patients with renal failure) Cardiac amyloid (covered under Restrictive Cardiomyopathies) Non-dynamic Limitations/Technical Considerations HCM can be mimicked- continued Pheochromocytoma Friedreich’s ataxia Noncancerous (benign) tumor in adrenal gland releasing adrenaline Rare cause of HTN. Concentric LVH (20%) Autosomal recessively inherited spinocerebellar degeneration Concentric LVH (most common) Decreased function Asymmetric septal thickening (uncommon) Increased RV mass (uncommon) Inferior MI with Previous LVH Limitations/Technical Considerations Coexisting aortic stenosis Accurate measure of pressure drop may not be possible AS and LVH may present with dynamic subaortic obstruction only after the aortic valve replacement HCM that is “unmasked” by the afterload reduction of the valve replacement Clinical Utility Diagnosis and Screening Echo: procedure of choice for accurate diagnosis of HCM Inherited disorder: screening is indicated for all first degree relatives of the affected individual even in asymptomatic due to high risk of sudden death with exertion Evaluation of Medical Therapy Echo: used to assess impact of medical therapy specifically diastolic function but also dynamic outflow obstructions Clinical Utility - continued On right: contrast in septum defining area perfused by the septal branch that will be injected Non-dynamic Monitory of Percutaneous Alcohol Septal Ablation Patient selection Extent, distribution and curvature of septal hypertrophy Cath Lab Catheter in septal coronary branch, contrast is injected during echo to show specific location before delivering of the agent Monitoring procedure in cardiac catheterization laboratory: baseline and post procedure Doppler data are used in conjunction with invasive hemodynamics to assess the reduction in the outflow obstruction Clinical Utility - continued Surgical Therapies Interoperative monitoring of myotomy allows evaluation of the adequacy of the procedure Non-dynamic Before and After Myectomy Prior Post 17-67a 17-67b Feigenbaum Feigenbaum Review of HCM Septal Hypertrophy Type I 17-45a Feigenbaum Septal Hypertrophy Type I – Same Patient 17-45b Feigenbaum HCM Type III with Massive Septal Hypertrophy and SAM 17-49a-49b Feigenbaum HCM Type III, Same Patient 17-50a-50b Feigenbaum Apical Hypertrophy Type IV 03-004b Feigenbaum Left Ventricular Opacification Agent Apical Hypertrophy Type IV 04-027b Feigenbaum No Classification. Focalized Hypertrophy Inferior Wall Only 17-51 Feigenbaum Pulmonary Hypertension with RVH Mimicking HCM Hypertrophy: right ventricular trabeculation True septal dimension noted by the double-headed arrow, septal and trabeculation dimension noted by the two inwardpointing arrows. 17-66 Feigenbaum Additional Notes on Hypertrophic Cardiomyopathies IHSS/ASH WITH SAM/HOCM Idiopathic hypertrophic subaortic stenosis = LVOT obstruction Shape of CW Doppler tracing in HOCM is described as “dagger” Restrictive Cardiomyopathy (RCM) Basic Principles Normal LV systolic fxn Impaired diastolic fxn dt a stiff, hypertrophied LV Heart failure symptoms are due: Resultant elevation in LV end-diastolic mmHg Inability to increase CO w/exercise dt impaired diastolic filling Heart Failure Definition Congestive heart failure Life-threatening condition Inability to maintain a normal cardiac output Can occur with normal systolic function Heart Failure and Restrictive Cardiomyopathy (RCM) RCM Right-sided failure predominates Initially with symptoms of peripheral edema and ascites Restricted Cardiomyopathy (RCM) History Congestive Heart Failure (CHF) Dyspnea Paroxysmal nocturnal dyspnea Orthopnea Fatigue Weakness Anorexia Angina Poor exercise tolerance Restrictive Cardiomyopathy Infiltrative Amyloidosis Hemochromatosis Glycogen storage disease Inflammatory Sarcoidosis Idiopathic (Löffler’s) Hypereosinophilic syndrome Restrictive - Infiltrative Infiltrative - Amyloidosis Multi-system disease of unknown cause Extracellular deposition of amyloid protein in the kidney, heart, liver, nerve, skin and tongue Results in tissue damage and organ malfunction 2D “Ground Glass” appearance (may also be seen in renal failure) Concentric LV and RV thickening May have asymmetric septal thickening (mimics HCM) Infiltrative – Amyloidosis continued Segmental wall motion abnormalities: common (septum and inferior wall) Global LV systolic function may be normal (early) or decreased (late) Usual presentation Male >40 years old with progressive biventricular failure Biopsies Amyloid found in myocardium, endocardium, pericardium and walls of intramural coronary arteries within the conduction system Infiltrative - Amyloidosis Classic cardiac amyloidosis. Note the uniform hypertrophy of the walls with abnormal myocardial texture. Myocardium: substantially brighter and may take on a speckled appearance (ground-glass) Secondary biatrial enlargement 17-39a Feigenbaum Infiltrative –Amyloidosis Same Patient 17-39b Feigenbaum Infiltrative - Hemochromatosis Infiltrative - Hemochromatosis Iron storage disease: Affects multiple organ and tissue systems w Iron May result in tissue damage and organ malfunction stored within the cardiac cell rather than extracellular Types Primary (idiopathic) Secondary Iron overload due to multiple blood transfusions in patients with chronic anemia, abnormal erythropoiesis Alcohol liver disease Infiltrative - Hemochromatosis Primary Clinical presentation: usually male >40 years with evidence of clinical tetrad (DM, liver, skin, and heart dz, with 1/3 manifesting congestive heart failure) “Bronze-diabetes” Liver function abnormalities CHF Infiltrative - Hemochromatosis Primary Chest x-ray: cardiomegaly Cardiac cath: increased LV filling pressures and reduced ventricular function (global or segmental) Echo/M-mode: LV cavity normal size to dilated Normal LV and RV wall thickness Dilated Cardiomopathy Infiltrative - Hemochromatosis Secondary Physical/History High output cardiac failure from anemia M-Mode/2D Increased LV wall thickness LV dilation Left atrial dilation May demonstrate normal or depressed systolic LV function with depressed fx indicating a worse prognosis Infiltrative - Hemochromatosis Parasternal short-axis view recorded in a patient with documented cardiac hematochromatosis. Note the increased wall thickness and the abnormal myocardial texture with modest reduction in systolic function. 22-34 Feigenbaum Infiltrative – Glycogen Storage Disease (Pompe’s) Infiltrative – Glycogen Storage Disease (Pompe’s) A group of inheritable disorders of glycogen metabolism, resulting from a specific enzymatic defects Echo Severe thickening of the IVS, free wall, and posterior left ventricle wall Tumor-like appearance of the papillary muscles Small left ventricular cavity Poor global left ventricular systolic function Inflammatory Inflammatory - Sarcoidosis Multi-system granulomatous disease; Involves the heart in about 25% of cases Females:males 2:1 Unknown etiology Progressive heart failure Diffuse fibrosis of the lungs/pulmonary hypertension Can cause sudden death due to electrical dysrhythmias Inflammatory - Sarcoidosis Echo/M-mode Increased LV wall thickness (early) Asymmetric septal hypertrophy LV dilation with decreased EF (late) Pericardial effusion MVP RV dysfunction due to pulmonary hypertension Diagnosis by echo is difficult. Suspect when a young pt presents w/increased LV dimensions, segmental wall motion abnormalities and/or ventricular aneurysms Inflammatory - (Löffler’s) Hypereosinophilic Syndrome Systemic illness characterized by persistently elevated blood eosinophil counts Unknown etiology Leads to endomyocardial/myocardial fibrosis and thrombosis Chest X-ray: cardiomegaly, linear calcification along left heart border (calcification of endocardial thrombus) dense fibrosis of ventricle in a postmortem dissected heart Inflammatory - (Löffler’s) Hypereosinophilic Syndrome Echo: Apical obliteration, Global LV systolic function May extend basally to the inflow portion of the ventricular wall Usually well preserved (including apical wall) Biatrial enlargement Posterior atrioventricular valves and subvalvular apparatus May become involved: massive mitral and/or tricuspid regurgitation Inflammatory –Loeffler’s Hypereosinophilic syndrome and obliteration of the LV apex. Homogeneous apical mass 22-35a Feigenbaum Review of Cardiomyopathies Hypertensive Heart Disease Hypertensive Heart Disease End-organ consequence of systemic hypertension Chronic systemic pressure overload Initially Left ventricular hypertrophy Diastolic function is impaired Systolic function is normal Long standing-chronic Systolic and diastolic dysfunction Ventricular dilation Hypertensive Heart Disease Typical echocardiographic findings Left ventricular hypertrophy Diastolic dysfunction Aortic root dilation Aortic valve sclerosis Mitral annular calcification Left atrial enlargement Non-dynamic Atrial fibrillation Hypertensive Heart Disease Echocardiographic approach Standard views Symmetrically increased wall thickness Increased end-diastolic wall thickness (>11 mm) Non-dilated/dilated chamber LV mass calculations Diastolic function Hypertensive Heart Disease Diastolic function Often first evidence of end-organ damage Antedating clear evidence of anatomic hypertrophy Impaired early diastolic dysfunction Prolonged IVRT E/A ratio reduction Prolonged deceleration time Hypertensive Heart Disease Systolic function Preserved early in disease course Mid cavity obliteration May occur in small, hypertrophied LV with normal function Associated Doppler velocity curve Brief, late-systolic high-velocity signal Duration of signal is shorter than with HCM Level of obstruction is mid-cavitary http://www.echojournal.org/vid eo/58/Ridiculous-LVH-1-of-3 Hypertensive Heart Disease Mid cavity obliteration Associated Doppler velocity curve Brief, late-systolic high-velocity signal Duration of signal is shorter than with HCM Level of obstruction is mid-cavitary Hypertensive Heart Disease Additional Echocardiography Findings Aortic root dilation Associated with increased tortuosity of the ascending aorta, arch, and descending aorta May have increased irregular echogenicity of the aortic walls Represents atherosclerosis Mitral annular calcification common in chronic hypertension (HTN) Left atrial pressure Combination of chronically elevated LV end-diastolic pressure and MR Non-dynamic Hypertensive Heart Disease Non-dynamic Limitations/Technical Considerations LV mass measurements Adequate endocardial and epicardial border definitions 2D vs. M-mode optimal controversial Differentiation of restrictive and hypertrophic heart disease Clinical Utility LV mass Strong predictor of clinical outcome in patient with hypertension Borderline hypertension: Degree of LVH reflects the chronic elevation of systemic pressure Index of the temporally average blood pressure over long periods LV mass may be more accurate for assessing the severity of hypertension than occasional BP recordings Left Ventricular Mass Determined by the left ventricular muscle volume and specific gravity of the muscle LV muscle volume Equal to total ventricular volume contained within the epicardial boundaries of the ventricle minus the chamber volume contained by the endocardial surfaces Left Ventricular Mass LV mass is calculated by Multiplying the left ventricular muscle volume by the specific gravity of muscle (1.04 g/ml) specific gravity n. (Abbr. sg, sp gr) The ratio of the mass of a solid or liquid to the mass of an equal volume of distilled water at 4°C (39°F) or of a gas to an equal volume of air or hydrogen under prescribed conditions of temperature and pressure. Left Ventricular Mass Penn-Cube Method LV measurements are made during diastole just below the tips of the mitral valve LV mass = 1.04 [(LVID + IVST + PWT)3 – (LVID)3 ] – 13.6 1.04 = specific gravity of myocardium g/ml IVST=Interventricular septal thickness (cm) LVID = Left ventricular internal diameter (cm) PWT= Posterior wall thickness (cm) Left Ventricular Mass ASE Method Overestimate LV mass by approximately 25% Regression equation has been derived for the calculation based on ASE method LV Mass = 1.04([LVID +PWT+IVST]3 – LVID3) *0.8 + 0.6 1.04 = specific gravity of myocardium g/ml IVST=Interventricular septal thickness (cm) LVID = Left ventricular internal diameter (cm) PWT= Posterior wall thickness (cm) Left Ventricular Mass Medical Therapy Monitor efficacy of medical therapy using echocardiographic measurements Effective antihypertensive therapy should reverse end-organ changes (i.e. result in regression of LVH) Evaluation of Heart Failure Symptoms Chronic hypertension Heart failure symptoms may be due to Diastolic or systolic left ventricular dysfunction Superimposed coronary artery disease Superimposed valvular disease Hypertensive hypertrophic cardiomyopathy Extreme form of preserved LV systolic function and heart failure symptoms Normal to hyperdynamic LV systolic function Concentric LVH Diastolic dysfunction Midventricular late systolic gradient d/t cavity obliteration Technically not a cardiomyopathy Evaluation of Heart Failure Symptoms Chronic hypertension Impairment of LV contractility can occur Even in absence of LV contractility can occur End-stage hypertensive heart disease has echocardiographic appearance of end-stage cardiomyopathy Evaluation of the PostCardiac-Transplant Patient Post-Cardiac-Transplant Basic Principle Three goals 1. 2. 3. Assessment of cardiac anatomy and physiology prompted by specific clinical problem The elusive goal of noninvasive diagnosis of early rejection of the transplanted heart Diagnosis of post-transplant coronary artery disease Post-Cardiac-Transplant Clinical Challenges Pericardial effusion Particularly early postoperative RV systolic dysfunction due to: Inadequate myocardial preservation at the time of transplantation Persistently elevated pulmonary vascular resistance Transplant rejection LV systolic dysfunction due to: Inadequate myocardial preservation Acute rejection early after transplantation Superimposed coronary artery disease at a longer interval after transplantation Post-Cardiac-Transplant Normal Findings Normal RV and LV size Wall thickness Systolic function Valvular anatomy Trivial MR, TR, and PR Abnormal septal motion Anterior motion of septum in systole Slight decrease in extent of systolic thickening of septum Post-Cardiac-Transplant :Normal Findings Suture lines in aorta and pulmonary artery: difficult to assess Small pericardial effusion in early postoperative period Rarely persists beyond a few weeks Pericardial effusions often loculated dt postoperative pericardial adhesions May have some degree of persistent elevation of pulmonary artery pressures Bi-atrial enlargement-if anastomosis of nl and donor atrium If anastomosis is of SVC and IVC for RA AND cuff of tissue with pulmonary veins for LA = little bi-atrial enlargement (BAE) and suture lines may not be evident Post-Cardiac-Transplant Abnormal Findings Acute rejection Increased LV mass Decreased systolic function Increase in echogenicity of the myocardium New or increasing pericardial effusion Post-Cardiac-Transplant Abnormal Findings Mild/Early rejection 2D echocardiographic changes are subtle Not accurate or reproducible enough for adjustments of medications Diastolic dysfunction seen with Doppler Post-Cardiac-Transplant Abnormal Findings Proposed echocardiographic approaches Diastolic function measurements using patient as baseline Decreased pressure half-time (increased early diastolic deceleration slope) Decreased isovolumetric relaxation time Increased E velocity Significant changes >20% E velocity >15% for pressure half-time >15% isovolumetric relaxation time Post-Cardiac-Transplant Abnormal Findings Transplant coronary artery disease Disease is often accelerated relative to CAD in non-transplant patients Epicardial vessels and microvasculature or diffusely involved Stress echocardiography=higher prevalence of false-negative Diffuse disease process masks regional wall motion abnormalities Dobutamine stress echocardiography = more accurate in this patient population Post-Cardiac-Transplant Limitations/Technical Considerations/Alternate Approaches Standard evaluation for rejection: transvenous endomyocardial biopsy Some centers utilize echocardiography guidance Bi-Atrial Enlargement (BAE) Several years post cardiac transplantation. Biatrial enlargement Echo density along the atrial septum, which represents a prominent atrial suture line Residual right ventricular and right atrial dilation are also present 19-69 Feigenbaum Left Ventricular Assist Device (LVAD) Heartmate II Pulmonary Heart Disease Pulmonary Heart Disease Chronic vs. Acute Chronic pulmonary heart disease Results in group of clinical signs and symptoms called cor pulmonale Underlying pathophysiology of cor pulmonale Chronic pressure overload of the RV ejecting into a highresistance pulmonary vascular bed Pulmonary Heart Disease Chronic vs. Acute Chronic pulmonary heart disease Results in group of clinical signs and symptoms called cor pulmonale Initially compensatory RVH with preserved RV function Progressively RV systolic function deteriorates leading to RV enlargement (RVE), moderate to severe TR and consequent right atrial enlargement (RAE) Pulmonary Heart Disease Chronic vs. Acute Acute pulmonary embolism Sudden onset of elevated pulmonary vascular resistance Pulmonary Heart Disease Echocardiographic approach Non-invasive evaluation of pulmonary artery pressures Tricuspid regurgitation VSD and PDA assessment Pulmonic regurgitant Doppler curve M-mode through pulmonic valve Right ventricular hypertrophy Dilation of right ventricle Ventricular septal motion : “paradoxical” Pulmonary Heart Disease Echocardiographic approach Non-invasive evaluation of pulmonary artery pressures Tricuspid regurgitation Estimation of Cardiac Pressures Systolic Right Heart Pressures Normal right heart pressures 25/10 mmHg 0-5 mmHg 25/5 mmHg Estimation of Cardiac Pressures Systolic Right Heart Pressures Tricuspid regurg. + right atrial pressure = Systolic RV Systolic BP – Ventricular septal defect = Systolic RV Systolic BP – patent ductus arteriosis = Systolic RV IVC Reactivity Estimation of Right Atrial Pressure IVC Reactivity Estimation of Right Atrial Pressure Inferior vena cava (IVC) view. Measurement of the IVC. The diameter (solid line) is measured perpendicular to the long axis of the IVC at endexpiration, just proximal to the junction of the hepatic veins that lie approximately 0.5 to 3.0 cm proximal to the ostium of the right atrium (RA). http://www.asecho.org/files/rhfinal.pdf IVC Reactivity Estimation of Right Atrial Pressure 4V2 + right atrial pressure = Systolic right heart pressure Ventricular Septal Defect Patent Ductus Arteriosus Estimation of Pulmonary Artery End Diastolic Pressure Normal 10 mmHg Pressure gradient between the pulmonary artery and the right ventricle during diastole Mean Pulmonary Artery Pressure Normal 16 mmHg Estimated using Peak pulmonary regurgitant velocity and From the right ventricular artery acceleration time Pulmonary Heart Disease Indirect signs M-mode High specificity (>90%) Low sensitivity (30-60%) Note:Absence of A wave Pulmonary hypertension Normal Pulmonary Heart Disease Doppler Rapid early systolic flow with abrupt midsystolic deceleration of flow indicates severe pulmonary hypertension Pulmonary HTN vs. Vol Overload Pulmonary Heart Disease Right Ventricular Pressure Overload Hypertrophy Dilation 07-049 Feigenbaum Pulmonary Heart Disease Right Ventricular Pressure Overload RVH on right ventricular free wall best seen in subcostal four chamber 07-056 Feigenbaum Pulmonary Heart Disease Right Ventricular Pressure Overload Dyskinetic septal motion 07-058a Feigenbaum Pulmonary Heart Disease Right Ventricular Pressure Overload Characterized by systolic flattening of interventricular septum reversed in late diastole 07-058b Feigenbaum Pulmonary Heart Disease Right Ventricular Pressure Overload 07-060a Feigenbaum Pulmonary Heart Disease Note Heavily trabeculated RV Dilated RV and RA RVH Pericardial effusion McConnell Sign Observed in acute pulmonary embolism http://www.echojournal.org/video/132/McC onnells-Sign-RV-dysfunction-inpulmonary-embolus 07-061 Feigenbaum Pulmonary Heart Disease Long standing pulmonary hypertension or acute pulmonary hypertension (PHTN) RV systolic dysfunction with secondary dilation Dilation: compensatory mechanism to maintain forward stroke volume Tricuspid regurgitation due to annular dilation and malalignment of papillary muscles (pap msc) Leads to further dilation and malalignment of papillary msc Tricuspid regurgitation leads to RA enlargement dt increased pressure and volume Pulmonary Heart Disease Secondary Tricuspid Regurgitation Careful attention to rule out other etiologies of TR Vegetation Rheumatic Carcinoid Ebstein’s anomaly Severe tricuspid regurgitation results in retrograde flow in hepatic and inferior vena cava Pulmonary Heart Disease Secondary Tricuspid Regurgitation Severe tricuspid regurgitation 12-029a Feigenbaum Pulmonary Hypertension Tricuspid Regurgitation Severity Pulmonary Hypertension Limitations/Technical Considerations Evaluation of cor pulmonale Poor ultrasound resolution resultant of obscuring hyperexpanded lung tissue Assessment of tricuspid regurgitation for qualification of pulmonary artery pressure dependent upon intercept angle Absence of TR does not translate to normal pulmonary pressures Overestimation of tricuspid regurgitation Graham Steell’s Murmur High-pitched and blowing murmur during early diastole with a decrescendo configuration Noted over the left upper-to-left midsternal area Result of high-velocity regurgitant flow across an incompetent pulmonic valve May be present during the whole of diastole Due to pulmonary-to-right ventricular pressure gradient throughout this time period Typically occurs in severe PHTN when the PA systolic pressure is >60 mm Hg Tricuspid Regurgitation Overestimation Avoid overestimation Measure outer edge of spectral Doppler and not spectral broadening at peak velocity resultant from transit time effect Pulmonary Heart Disease Clinical Utility Confirmation of clinical diagnosis of cor pulmonale in patient with right ventricular failure and chronic lung disease Essential to exclude other causes of pulmonary hypertension Atrial septal defect Mitral regurgitation Noninvasive measurements of pulmonary pressure used for assessment of medical therapy routinely used http://www.amicusvisua lsolutions.com/obrasky/ 06029_01W.jpg Pulmonary Heart Disease Clinical Utility-continued Acute pulmonary embolism May visualize residual thrombus originating from or in transit in the right side of the heart (DVT) TEE/TTE imaging can demonstrate thrombi in main, right or left pulmonary arteries Sensitivity is low Usually thrombi are located more distally in pulmonary vasculature Adequate visualization of pulmonary artery bifurcation in TTE is not possible in all patients due to interposition of the air-filled trachea and bronchi Pulmonary Heart Disease Clinical Utility-continued 22-45 Feigenbaum Pulmonary Heart Disease Clinical Utility-continued Indirect signs of pulmonary embolism Elevated pulmonary artery pressures Evidence of acute right ventricular pressure overload “D-shaped” PSAX-PM level Right ventricular dilation and dysfunction Non-dynamic images http://www.4ventavis.com/img/ patient/diagnosingpah_05.jpg Pulmonary Heart Disease Clinical Utility-continued Indirect signs of pulmonary embolism Tricuspid regurgitation Similar findings in patient w/chronic recurrent pulmonary emboli Reasons for study may be: Chest pain, dyspnea or heart failure Pulmonary Heart Disease- Pulmonary Emboli Alternate Approaches Cardiac catheterization allows direct Measurement of RV and PA pressures Calculation of pulmonary vascular resistance Angiography can evaluate RV size and systolic function utilizing contrast Standard approach Radionuclide lung ventilation-perfusion scan Sources As noted in individual slides and the following: Feigenbaum H, Armstrong W. (2004). Echocardiography. (6th Edition). Indianapolis. Lippincott Williams & Wilkins. Goldstein S., Harry M., Carney D., Dempsey A., Ehler D., Geiser E., Gillam L., Kraft C., Rigling R., McCallister B., Sisk E., Waggoner A., Witt S., Gresser C.. (2005). Outline of Sonographer Core Curriculum in Echocardiography. Otto C. (2004). Textbook of Clinical Echocardiography. (3rd Edition). Elsevier & Saunders. Reynolds T. (2008). The Echocardiographer's Pocket Reference. (3rd Edition). Arizona. Arizona Heart Institute.