Right Heart Catheterization and Pulmonary Hypertension (Athens Oct 2024) PDF

RIGHT HEART CATHETERIZATION AND THE DIAGNOSIS OF PULMONARY HYPERTENSION Professor Konstantinos Dimopoulos MD MSc PhD FESC Consultant Cardiologist Professor of Practice in ACHD and PH Adult Congenital Heart Centre and Centre for Pulmonary Hypert...

RIGHT HEART CATHETERIZATION AND THE DIAGNOSIS OF PULMONARY HYPERTENSION Professor Konstantinos Dimopoulos MD MSc PhD FESC Consultant Cardiologist Professor of Practice in ACHD and PH Adult Congenital Heart Centre and Centre for Pulmonary Hypertension Royal Brompton Hospital and Imperial College London, UK ▪ 40y old ▪ Progressive SOB over last 2 years ▪ Now can manage less than 50m on flat ▪ Ankle swelling, abdominal swelling ▪ Mother, grandmother died in their 30s-40s IHD? DCM/Heart failure? Lungs?........ 5 groups of PH  Pathogenesis of PAH Normal Abnormal ▪ Increased pulmonary vascular resistance and raised pulmonary artery pressure: ⬧ Vascular proliferation and remodelling ⬧ Distal muscularisation of normally non-muscular arteries ⬧ Increased muscularisation of muscular pulmonary arteries ⬧ Neointima formation ⬧ Formation of plexiform lesions ACT 08/1308 October 09 Jeffery T, Morrell N. Prog Cardiovasc Dis 2003;45:173–202  Basis of grades of hypertensive PVD found in association with large VSDs and functionally related diseases Grade of hypertensive pulmonary vascular disease 1 2 3 4 5 6 None Type of intimal Cellular reaction Fibrous & fibroelastic Plexiform lesion State of media of Hypertrophied arteries and Some generalized dilatation arterioles Local “dilatation lesions” Pulm hemosiderosis Necrotizing arteritis Heath, Edwards. Circulation 1958  The malignant nature of PAH Smooth muscle 1 Intimal proliferation & fibrosis 1 dystrophy Thrombosis Early intimal Medial Medial proliferation hypertrophy hypertrophy Plexiform lesions Intimal thickening Narrowing of lumen Increased wall thickness Plexiform lesion ▪ Angiogenesis & evasion of apoptosis2 ▪ Self-sufficient in growth signals & insensitivity to anti- Hallmarks shared growth signals2 with cancer ▪ Tissue invasion and limitless replicative potential2 1. Gaine S. JAMA 2000; 284:3160-8. 2. Rai PR, et al. Am J Respir Crit Care Med 2008; 178:558-64.  The rapidly progressive nature of PAH Disease is often advanced before it is apparent 1. REVERSIBLE IRREVERSIBLE Smooth muscle Intimal proliferation & fibrosis Adventia dystrophy Thrombosis Media Early intimal Medial Intima proliferation hypertrophy Plexiform lesions Pre- Symptomatic / Declining / decompensated 2. symptomatic decompensating CO Symptom threshold RV DYSFUNCTION PAP PVR WHO FC I WHO FC II - III WHO FC IV Time 1. Gaine S. JAMA 2000; 284:3160-8. 2. Domenighetti G, et al. Swiss Med Wkly 2007; 137:331-6.  Cardiopulmonary Physiology VCO2 PERIPHERAL V/Q CO=HRxSV Hb TISSUES AIR CO2 O2 LUNG LUNG BLOOD/ HEART VENTILATION PERFUSION PERIPHERY MUSCLE VO2 Obstruction iPAH CHF Anaemia Restriction R-L shunt CHD PVD Myoskeletal PE Valve disease Skeletal muscle CHF Obesity Detraining Endothelial dysfunction  Prognosis is extremely poor in PAH Median survival 2.8 years 100 68% 80 48% Survival (%) 60 34% 40 20 0 0 0.5 1 1.5 2 2.5 3 3.5 4 4.5 5 Follow-up (years) D’Alonzo GE, et al. Ann Internal Med 1991; 115:343-9. Survival in patients with untreated PAH of different aetiologies 1 0.9 surviving untreated PAH 0.8 CHD Proportion of patients 0.7 0.6 0.5 IPAH 0.4 CTD 0.3 0.2 HIV 0.1 0 0 1 2 3 4 5 Time (years) McLaughlin VV, et al. Chest 2004; 126:78S-91S.  A small delay in diagnosis has a dramatic impact on prognosis Advanced lung cancer1 WHO Class IV IPAH2 6 months Advanced colorectal cancer1 Advanced breast cancer1 WHO class III IPAH2 2.6 years WHO class I-II IPAH2 4.9 years Ischaemic cardiomyopathy3 0 1 2 3 4 5 6 7 Survival (years) 1. Kato I, et al. Cancer 2001; 92:2211-9. 2. D'Alonzo GE, et al. Ann Intern Med 1991; 115:343-9. 3. Felker GM, et al. N Engl J Med 2000; 342:1077-84. FRENCH REGISTRY: CTD and CHD ARE THE LEADING CONDITIONS ASSOCIATED WITH PAH Idiopathic 39% Connective tissue diseases 15% Congenital heart diseases 11% Portal hypertension 10% Appetite suppressant exposure 9.5% HIV infection 6% Familial 4% (% of population) 2 RISK FACTORS 4% ACT 08/1308 October 09 Humbert M, et al. AJRCMM 2006; 173:1023–30.  Incidence and prevalence of PAH ▪ IPAH: ⬧ ~1–8 individuals per million per year1,2 ▪ PAH-CTD (SSc): ⬧ affects ~ 8-12% of CTD patients3,4 ▪ Prevalence of PAH associated with CHD:  10% of adults with CHD5 ▪ Prevalence of all types of PAH is 30-50/m/y2 1. Taichman DB, et al. Clin Chest Med 2007; 28:1-22. 2. Peacock AJ, et al. Eur Resp J 2007; 30:104-9. 3. Hachulla E, et al. Arthritis Rheum 2005; 52:3792-800. 4. Mukerjee D, et al. Ann Rheum Dis 2003; 62:1088-93. 5. Duffels MGJ, et al. Int J Cardiol 2006; doi:10.1016/j.ijcard.2006.09.017:1-7.  Finding a needle in a haystack ▪ PAH is frequently asymptomatic until it has reached an advanced stage and prognosis is poor ▪ Symptoms of PAH can be subtle and overlap with other disorders Asthma? Symptoms2 BREATHLESSNESS Left ventricular disease? Fatigue Weakness COPD? Pulmonary hypertension? Gibbs JSR. Eur Respir Rev 2007; 16:8-12. Rich S, et al. Ann Intern Med 1987; 107:216-23.  PAH Diagnosis – a high index of suspicion needed ▪ Symptoms initially insidious and nonspecific ▪ Diagnosis should be considered in any patient with: ⬧ unexplained dyspnea on exertion ⬧ fatigue, or exercise limitation ⬧ clinical signs consistent with right-heart dysfunction ⬧ patients with family history of pulmonary hypertension ⬧ conditions generally associated with high prevalence of PAH Levine DJ. Respiratory Care 2006;51(4):368–381  Diagnosis of PAH is typically delayed ▪ Low prevalence1,2 ▪ Low suspicion3 DIAGNOSIS IS ▪ Asymptomatic in early TYPICALLY stages4 DELAYED BY ▪ Non-specific symptoms3 ≥ 2 YRS3 1. Taichman DB, et al. Clin Chest Med 2007; 28:1-22. 2. Peacock AJ, et al. Eur Resp J 2007; 30: 104-9. 3. Gibbs JSR. Eur Respir Rev 2007; 16:8-12. 4. Barst R, et al. JACC 2004; 43: 40S–47S.  The UK and EIRE specialist centres ▪ Glasgow: Western Infirmary ▪ Dublin: Mater Misericordiae ▪ Cambridge: Papworth ▪ Newcastle: Freeman ▪ Sheffield: Royal Hallamshire ▪ London Centres: ⬧ Royal Free ⬧ Royal Brompton ⬧ Great Ormond Street ⬧ Hammersmith Gibbs S, et al. Heart 2008; 94 (Suppl 1):i1-41.  Definition of PH 35 mean PAP (mmHg) 30 32 25 S 20 17 15 13 10 D 5 4 0 Normal resting PAH resting ▪ Old PH definition: mPAP>25 mmHg at rest Galiè N, et al. Eur Heart J 2009; 30:2493-2357.  ∆𝑃 = 4𝑉 2 Modified Bernoulli equation PAPsyst=TRgradient+JVP PAPsyst=4V2+JVP RV LV RV RA LA RA Pre- Symptomatic / Declining / decompensated 2. symptomatic decompensating CO Symptom threshold SVC RV DYSFUNCTION IVC PAP PVR WHO FC I WHO FC II - III WHO FC IV Time !!But, if RV impaired, gradient will not rise!!! 1. Gaine S. JAMA 2000; 2 2. Domenighetti G, et al. Swiss Med Wkly 2007; Echocardiographic variables suggestive of PH Peak tricuspid Presence of other echo Echocardiographic regurgitation velocity ‘PH signs’a probability of pulmonary (m/s) hypertension ≤2.8 or not measurable No Low ≤2.8 or not measurable Yes Intermediate 2.9–3.4 No 2.9–3.4 Yes High >3.4 Not required Use TR gradient and additional signs of PH to estimate probability of PH. Refer to expert/cath if intermed/high  Exclude: LHD Lung disease thromboembolic disease ECHO, V/Q, Expert referral Images courtesy of Joanna Pepke-Zaba  RHC is the only way to make a definitive diagnosis of PAH 100 r2=0.4515 pressure difference Trans-Tricuspid 80 (echo) (mmHg) False 60 positive 40 20 False negative 0 0 20 25 40 60 80 mPAP (mmHg) measured invasively (RHC) 1. Gibbs JSR. Eur Respir Rev 2007; 16:8-12. 2. Mukerjee D, et al. Rheumatology 2004;43:461-6. Diagnosis, prognostication and treatment depend on RHC When? How often? RHC in risk stratification  Right heart cath NAV No DPG  Pulmonary and systemic vascular resistance I I Qp Ao Qs PA LA RA 𝑉 𝑅= R R 𝐼 RV LV V V 𝑇𝑟𝑎𝑛𝑠𝑝𝑢𝑙𝑚𝑜𝑛𝑎𝑟𝑦 𝑔𝑟𝑎𝑑𝑖𝑒𝑛𝑡 𝑚𝑒𝑎𝑛 𝑃𝐴 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 − 𝑚𝑒𝑎𝑛 𝐿𝐴 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 𝑃𝑉𝑅 = = 𝑄𝑝 𝑄𝑝 𝑇𝑟𝑎𝑛𝑠𝑝𝑢𝑙𝑚𝑜𝑛𝑎𝑟𝑦 𝑔𝑟𝑎𝑑𝑖𝑒𝑛𝑡 𝑇𝑟𝑎𝑛𝑠𝑝𝑢𝑙𝑚𝑜𝑛𝑎𝑟𝑦 𝑔𝑟𝑎𝑑𝑖𝑒𝑛𝑡 𝑃𝑉𝑅𝐼 = = 𝑄𝑝 𝑖𝑛𝑑𝑒𝑥𝑒𝑑 𝑄𝑝 𝐵𝑆𝐴 = 𝑃𝑉𝑅 × 𝐵𝑆𝐴 (𝑊𝑈 × 𝑚2 )൱ 𝑚𝑒𝑎𝑛 𝐴𝑜𝑟𝑡𝑖𝑐 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 − 𝑚𝑒𝑎𝑛 𝑅𝑖𝑔ℎ𝑡 𝑎𝑡𝑟𝑖𝑎𝑙 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 𝑆𝑉𝑅 = 𝑄𝑠  Pre versus postcapillary PH R=V/I TP gradient LV TPG=mPAP-mLAP Ao PVR=TPgradient/CO Normal RV PA RA LA RA RA LV RV Ao Pre- PA >20mmHg capillary 20mmHg >15mmHg capillary LA RA RA  Isolate postcapillary vs combined pre and post TP gradient R=V/I PVR=TPgradient/CO LV RV Ao PA Isolated >15mmHg Post- LA capillary RA RA RV PA LV Ao Combined Pre >15mmHg and Post- LA capillary RA RA No DPG  Pharmacological testing during RHC if PVR>2.5WU or TPG>12mmHg: MILRINONE, Na Nitroprusside TPG>12mm Hg OR PVR>2.5WU NO YES LUNGS LA SYSTEMIC CIRCULATION sodium NO nothing nitroprusside PVR≤3WU Ao DPG3WU DPG≥7mmHg Ao nitroprusside PA LV Courtesy of Prof A Kempny, RBH Combined Pre& TPG Postcapillary RV PH RA mPAP ≥ 25mmHg mPWP >15mmHg Dimopoulos et al. JACC Int 2018 Dimopoulos et al.. JACC Int 2018  Purpose of RHC: TPG, CO & PVR ▪ Measure pressures ▪ Measure (cardiac output) pulmonary blood flow Fick-oxygen method Thermodilution ▪ Vascular resistance: pulmonary ▪ Detect and measure Shunts ▪ Assess valve disease ▪ Assess coronary arteries Quality control Pressure Measurement Balancing Transducer zeroed at the midthoracic line in a supine patient, halfway between the anterior sternum and the bed surface= level of LA. 50 0  Pressure Measurement Balancing and Calibration ▪ Calibration  Mercury manometer attached to free port with 100 mm Hg of pressure transmitted through fluid-filled line  Provides accurate scaling of pressure measurement ▪ Balancing a transducer  Variable resistance is interpolated into circuit so that at an arbitrary baseline pressure the voltage across the output terminal can be reduced to zero  Zero reference  Midchest level (atria)  Measure antero-posterior thoracic diameter at angle of Louis Baim DS and Grossman W. Cardiac Catheterization, Angiography, and Intervention. 5th Edition. Baltimore: Williams and Wilkins, 1996.  Pressure Measurement Damping UNDER damped OPTIMALLY damped OVER damped Reverbrations Artifacts, overshooting above less responsive to sudden true pressure with sudden changes in pressure pressure changes Baim DS and Grossman W. Cardiac Catheterization, Angiography, and Intervention. 5 th Edition. Baltimore: Williams and Wilkins, 1996.  Right Heart Catheterization Right Atrial Pressure “a” wave – Atrial systole “c” wave – Protrusion of TV into RA “x” descent – Relaxation of RA – Downward pulling of tricuspid annulus by RV contraction “v” wave – RV contraction – Height related to atrial compliance & amount of blood return – Smaller than a wave “y” descent – TV opening and RA emptying into RV  Right Heart Catheterization Abnormalities in RA Tracing ▪ Elevated a wave  Tricuspid stenosis  Decreased RV compliance due to RV failure  Pulmonary hypertension ▪ Cannon a wave  A-V asynchrony (3rd degree AVB, VT, V-pacer) ▪ Absent a wave  Atrial flutter or fibrillation ▪ Elevated v wave  TR  RV failure  Reduced atrial compliance (restrictive myopathy) ▪ Equal a and v waves  Tamponade  Constrictive physiology https://wellcomecollection.org/works/htn7vsj Davidson CJ, et al. Cardiac Catheterization. In: Heart Disease: A Textbook of Cardiovascular Medicine, Edited by E. Braunwald, 5th ed. Philadelphia: WB Saunders Company, 1997 The jugular venous pulse, Paul Wood https://wellcomecollection.org/works/htn7vsjr  Pressure Measurement Wedge Pressure ▪ Wedge Pressure  end-hole catheter with its open end-hole facing a capillary bed, with no connecting vessels conducting flow into or away from the “designated” blood vessel between the catheter’s tip and the capillary bed  True wedge pressure can be measured only in the absence of flow, allowing pressure to equilibrate across the capillary bed 30 20 mmHg 10 0 No DPG  PV LA Static column of blood Wedge Pressure Air Zone 1: No Air Zone 2: No PA PV Impacts on Air PV PWP Zone 3 LA RV  Right Heart Catheterization Abnormalities in PCWP Tracing PCWP not equal to LV end diastolic pressure – Mitral stenosis – Atrial myxoma – Cor triatriatum – Pulmonary venous obstruction – Increased pleural pressure PV LA Davidson CJ, et al. Cardiac Catheterization. In: Heart Disease: A Textbook of Cardiovascular Medicine, Edited by E. Braunwald, 5th ed. Philadelphia: WB Saunders Company, 1997  RECOMMENDATION ON FLUID CHALLENGE FOR UNMASKING HFpEF ▪ Fluid challenge may be useful in identifying patients with occult HFpEF ▪ Current evidence suggests that administration of 500 ml of fluid over 5 to 10 min is safe and may help to distinguish patients with PAH from those with occult LV diastolic dysfunction. ▪ Must be interpreted with caution and should not be used alone to discard a diagnosis of PAH. Eur Respir J 2019; 53:1801897  “We maintain a strong Eur Respir J 2019; 53:1801897 recommendation against the use of PAH therapies in group 2 PH” No fluid challenge ? ? ? Postcapillary pulmonary hypertension: Treating the left heart ▪ Improve global management of the underlying condition:  Repair of valvular heart disease and  Aggressive therapy for HF  Nonspecific vasodilators (nitrates and hydralazine)  Optimize volume status (severe HF)  LV assist device may lower PAP (LV unloading) ▪ Avoid vasoreactivity testing ▪ There is no new evidence supporting the use of PAH therapies in PH-LHD  Shunt Detection & Measurement Oximetry Run: Look for step-up in sats PAPVR SV ASD AP window 2um ASD ALCAPA VSD  Steady state/rapid collection  Small shunts may be missed (Qp/Qs1.5  Shunt Detection & Measurement Oximetry Run: Look for step-up in sats 60% 100% 80% Qp 𝑄𝑝 𝑃𝐵𝐹 (𝑆𝑎𝑡𝑠𝐴𝑂 − 𝑆𝑎𝑡𝑀𝑉) = = 𝑄𝑠 𝑆𝐵𝐹 (𝑆𝑎𝑡𝑠𝑃𝑉 − 𝑆𝑎𝑡𝑠𝑃𝐴) 100% Qs 80% 60% 𝑄𝑝 (100 − 60) 40 = = =2 𝑄𝑠 (100 − 80) 20 60% 80% 100% 60%  Mixed venous sats SVC: closely approximates true systemic venous saturation PA RA 𝟑 × 𝑺𝑽𝑪𝒔𝒂𝒕𝒔 + 𝑰𝑽𝑪𝒔𝒂𝒕𝒔 𝑴𝑽𝒔𝒂𝒕𝒔 = 𝟒 RV IVC Phlamm Equation IVC: Highly saturated: kidneys receive 25% of CO and extract minimal oxygen  Oximetry Run Shunt Detection & Measurement SVC AO AO LUPV SVC SVC LA LLPV PA PA RA RA RA LV LV LV RV RV RV IVC IVC HV HV IVC HV 2um ASD PAPVD LUPV to innom vein  Cardiac Output Measurement Techniques 1. Fick-Oxygen Method, direct vs indirect 2. Indicator-Dilution Methods 1. Indocyanine Green 2. Thermodilution 3. CMR Direct Fick: Measuring VO2. This is the best way of estimating CO/PBF in the cath lab Thermodilution https://seattleclouds.com/myapplications/dukeg/ican/pacath.html  Cardiac Output Measurement Thermodilution Method Sources of Error (± 15%) – Unreliable in tricuspid regurgitation – Blood temperature in pulmonary artery may fluctuate with respiratory and cardiac cycles – Low cardiac output: warming of blood by walls of cardiac chambers and surrounding tissues: overestimation of cardiac output – Warming of injectate in syringe by the hand Baim DS and Grossman W. Cardiac Catheterization, Angiography, and Intervention. 5 th Edition. Baltimore: Williams and Wilkins, 1996.  Cardiac Output Measurement Law of concentration of mass: O2 breathed in… equals oxygen captured Fick Oxygen Method by blood crossing the lungs ▪ Fick Principle: The total uptake or release of any substance by an organ is the product of blood flow to the organ and the arteriovenous concentration difference of the substance. ▪ Applied to lungs, the substance released to the blood is oxygen, oxygen consumption is the product of arteriovenous difference of oxygen across the lungs and pulmonary blood flow. Oxygen consumption Qp = Arteriovenous O2 difference ▪ In the absence of a shunt, systemic blood flow Qs=Qp. Baim DS and Grossman W. Cardiac Catheterization, Angiography, and Intervention. 5th Edition. Baltimore: Williams and Wilkins, 1996.  VO2 O2 O2 A = 1.34× × 70% 70% AO SVC 98% 98% = PA 1.34× × RA LV RV IVC HV 𝑚𝑙 𝑙 𝑉𝑂 2 𝑚𝑖𝑛 𝑄𝑝 ( )= × 0.1 𝑚𝑖𝑛 𝑚𝑙𝑂2 𝑚𝑙𝑂2 𝐶𝑎𝑂2 − 𝐶𝑣𝑂2 𝑑𝑙𝑏𝑙𝑜𝑜𝑑 𝑑𝑙𝑏𝑙𝑜𝑜𝑑 CCM Medgraphics VO2: Direct Fick (more accurate than indirect) ▪ METABOLIC ASSESSMENT ▪ Measurements can be obtained with breath-by-breath analysis or user- defined averaging. ▪ Gas sensors measure both oxygen and carbon dioxide. ▪ Ventilator patients can be tested on elevated (above 60%) or fluctuating FiO2  Shunt Detection & Measurement Oximetric Methods ▪ Fick Principle: ASD mild-moderate PAH, Qp/Qs>2 AO  Pulmonary circulation 100% PA of blood=Hb x 1.36 x PA O sat O2 content 2 (Qp) utilizes PA and PV 80% VO2 TISSUES/PERIPHERY saturations AIR O2 O2  Systemic circulation (Qs) utilizes MV and 60% MV 100% PV O2 content of blood=Hb x 1.36 x LA O2sat Ao saturations Law of conservation of mass: what O2 inhaled= O2 reaching the tissues Why is there a different Δsats in lungs and in periphery? PBF>SBF 𝑂2 𝑐𝑜𝑛𝑡𝑒𝑛𝑡 = 1.34 × 𝐻𝑏 × 02 𝑠𝑎𝑡𝑠 + 0.003 ∗ 𝑃𝑎02 𝑉𝑂2 𝑉𝑂2 𝑄𝑝 = 𝑄𝑠 = 𝑃𝑉𝑂2 − 𝑃𝑎𝑂2 𝐴𝑜𝑂2 − 𝑀𝑉𝑂2  Pulmonary and systemic vascular resistance I R 𝑉 𝑅= 𝐼 V 𝑇𝑟𝑎𝑛𝑠𝑝𝑢𝑙𝑚𝑜𝑛𝑎𝑟𝑦 𝑔𝑟𝑎𝑑𝑖𝑒𝑛𝑡 𝑚𝑒𝑎𝑛 𝑃𝐴 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 − 𝑚𝑒𝑎𝑛 𝐿𝐴 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 𝑃𝑉𝑅 = = 𝑄𝑝 𝑄𝑝 𝑇𝑟𝑎𝑛𝑠𝑝𝑢𝑙𝑚𝑜𝑛𝑎𝑟𝑦 𝑔𝑟𝑎𝑑𝑖𝑒𝑛𝑡 𝑇𝑟𝑎𝑛𝑠𝑝𝑢𝑙𝑚𝑜𝑛𝑎𝑟𝑦 𝑔𝑟𝑎𝑑𝑖𝑒𝑛𝑡 𝑃𝑉𝑅𝐼 = = 𝑄𝑝 𝑖𝑛𝑑𝑒𝑥𝑒𝑑 𝑄𝑝 𝐵𝑆𝐴 = 𝑃𝑉𝑅 × 𝐵𝑆𝐴 (𝑊𝑈 × 𝑚2 )൱ 𝑚𝑒𝑎𝑛 𝐴𝑜𝑟𝑡𝑖𝑐 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 − 𝑚𝑒𝑎𝑛 𝑅𝑖𝑔ℎ𝑡 𝑎𝑡𝑟𝑖𝑎𝑙 𝑝𝑟𝑒𝑠𝑠𝑢𝑟𝑒 𝑆𝑉𝑅 = 𝑄𝑠 PAH-CHD: the most common type or PAH Case 1 PBF=4 l/min TPG=54-10=44mmHg 𝟓𝟒 − 𝟏𝟎 𝑷𝑽𝑹 = = 𝟏𝟏𝑾𝑼 𝟒  Case 2 ▪ Coarctation of the aorta. ▪ Aortic stenosis ▪ End to end Repair of coarctation of the aorta – 1970s ▪ Aortic valvotomy –Relief of subaortic stenosis with myotomy and wedge resection with homograft AVR,1970s ▪ Ross procedure for degenerative homograft – 1990s ▪ Pacemaker implantation in 1970s ▪ Persistent Atrial Tachycardia associated with heart failure, requiring DCCV ▪ SOB on exertion, FC 3  RA RV PA PAWP  TPG>12mm Hg OR PVR>2.5WU Postcapillary PH NO NO nothing YES sodium nitroprusside (SNP) CI25mmHg >15mmHg capillary LA Low PVR RA RA (no reactive component) RV LV Ao Post-capillary PH with PA a pre-capillary Post- >25mmHg >15mmHg component High PVR capillary LA RA RA (with reactive component) Diastolic pressure difference (DPD)=DiastolicPAP-meanPAWP  CXR 04/09/2018 Tetralogy of Fallot TOF repair with a transannular goretex patch PVR  RV RV RA ↑ ↑ LV RV/LV LV PA ↑ PAWP ↑ ↑ : “There were extremely dense pericardial adhesions, due in part to a thickened parietal pericardium, but there appeared to be definite evidence of constriction of the anterior part of the right ventricle and the right atrium, and around the SVC right atrial junction”