5 consecutive cases of PH I wish I never saw

Transcription

1 5 consecutive cases of PH I wish I never saw Abubakr A Bajwa. MD, FCCP Associate Professor of Medicine Division Chief Pulmonary, Critical Care and Sleep Medicine Director Pulmonary Hypertension and Interstitial Lung Disease Clinic (Not a Center of Comprehensive Care) yet University of Florida College of Medicine/Jacksonville

2 Case 1 62 y.o. male with PMH of COPD (on home oxygen at 4L/min), MI in 2002 (stent to LCx), Ventricular Fibrillation following MI in 2002 (s/p pacemaker), HLD, PFO, DM, Gout, OSA (on BiPAP at home for hrs of sleep), Former cigarette smoker who was transferred from Hospital following NSTEMI and AECOPD. LHC cath did not show any significant CAD

3 CTA chest negative for PE. Mild emphysema, bibasilar reticular opacities Scattered small LNs

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6 Initial RHC at OSH RAP 13 RV 81/16 PAP 81/36 PA mean 51 PCWP 14 CO 3.6 CI 1.8 PVR 10.2 WU/822 dynes SvO2 61% Aortic saturation 94%

7 Repeat RHC HR: 81 CVP 6 MAP: 88mmHg PA systolic: 68 mmhg PA diastolic: 24 mmhg PA mean: 39 mmhg PCWP: 7 mmhg BSA: 2.0 TPG (PAD-PAWP): 17 CO (thermodilution): 3.27L/min CI (thermodilution): 1.55L/min/m2 PVR (thermodilution): 807 dynes.sec/cm5 (10 wu) SVR (thermodilution): 2000dynes.sec/cm5 CO (Fick's): 2.71L/min CI (Fick's): 1.4 L/min/m2 PVR (Fick's): 946 dynes.sec/cm WOODS UNIT SVR (Fick's): 2003 dynes.sec/cm5 Chamber sats: Arterial: 85.3% Low SVC: 59 % PA: 54.7%

8 Repeat Echo 1. - Study Quality - Adequate Normal left ventricular size and wall thickness. Moderate global left ventricular dysfunction. Septal motion due to RV pressurevolume overload. Left ventricular ejection fraction ranges from 35-40% Moderate right ventricular enlargement. Hypokinetic wall motion and decreased function. Estimated RV systolic pressure is 72 mmhg, assuming right atrial pressure equals 5 mmhg consistent with severe pulmonary hypertension. 4. Tachycardia 120 BPM

9 Started on IV flolan RHC 12/2/14: MAP 75 /CVP 15 /PAs 118/ PAd 56/ PAm 76/ PCWP 16/ PVR 1277/ SVR 1590/ CO 3.5/ CI ng/kg/min flolan RHC 12/6/14 PAs 84, PAd 52/ PAm 63/ PCWP 16/ CO 3.59/ CI 1.67/ PVR 1048/ SVR 1344

10 Above 10 ng/kg/min he would get more SOB and more hypoxic despite PAOP never going above 16

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15 Due to worsening lung infiltrates and hypoxia despite being on Bpap decided to intubate, patient died of cardiac arrest immediately post intubation Family declined autopsy

16 Was this PVOD or PAH on TOP of systolic HF?

17 Case 2 65 with CAD s/p CABG latest EF by echo around 50% No significant CAD by L heart cath in sept 2013 He had a R heart cath done and it showed that his PCWP and LVEDP was 15 PA mean was about 48 and C.I was about 1.8, he was given nipride which improved his PA mean to 30s and improved C.O?? Subsequently he was started on Revatio 20 mg TID but clinically did not improve. He then saw Dr ABCD who stopped his sildenafil and started him on Norvasc He has WHO class 3 symptoms, He cannot lay flat unless he has his CPAP on which he uses regularly for his sleep apnea, He is on diuretics for LE edema but he feels more swelling in his abdomen than legs. He also has an ICD in place. He thinks the SOB started fairly acutely about 2 years ago.

18 RHC HR MAP - 95 mmhg RA - 20 mmhg PA systolic - 75 mmhg PA diastolic - 50 mmhg PA mean - 58 mmhg PCWP - 24 mmhg CO (thermodilution) L/min CI (thermodilution) L/min/m2 BSA 2.27 TPG (PAD-PCWP) - 34 PVR dyn.s/cm5 SVR dyn.s/cm5 Chamber sats Arterial % Low SVC % PA % CO (Fick's) L/min CI (Fick's) L/min/m2 PVR (Fick's) Dyn.s/cm5 SVR (Fick's) Dyn.s/cm5 Vasodilator Testing: After 20 ppm of NO for 20 min: HR - 98 MAP - 98 mmhg RA - 17 mmhg PA systolic - 66 mmhg PA diastolic - 45 mmhg PA mean - 52 mmhg PCWP - 24 mmhg CO (thermodilution) L/min CI (thermodilution) L/min/m2 BSA 2.27 TPG (PAD - PCWP) - 28 PVR dyn.s/cm5 SVR dyn.s/cm5

19 VQ scan Normal LFTs and hepatitis serologies normal or negative

20 Echo Study Quality: Adequate for Interpretation LV Function: There is mild to moderate global LV dysfunction. Septal motion due to RV pressurevolume overload and IVCD. LV Size/Thickness: Normal left ventricular size and wall thickness. Wall Motion: The basal inferolateral segment, and the mid inferolateral segment are hypokinetic. The apex is akinetic. The basal anteroseptum, basal inferoseptum, mid anteroseptum, and the mid inferoseptum are asynchronous. All other segments contract normally. Doppler Findings: Mild mitral regurgitation. Moderate to severe tricuspid regurgitation. Mild pulmonic regurgitation. Estimated RV systolic pressure is 51 mmhg, assuming right atrial pressure equals 15 mmhgâ consistent with moderate pulmonary hypertension.

21 Echo (contd) Right Ventricle: The right ventricle is markedly enlarged. Akinesis of the RV free wall is seen. An echogenic mass consistent with a catheter is seen. Hypokinetic wall motion and decreased function. Left Atrium: Mild left atrial enlargement. Right Atrium: Marked right atrial enlargement. An echogenic mass consistent with a catheter is seen. Aorta: Normal aorta. Pulmonary Artery: Normal pulmonary artery. Mitral Valve: Normal mitral valve. Aortic Valve: Normal aortic valve. Tricuspid Valve: Normal tricuspid valve. Pulmonic Valve: Normal pulmonic valve. Pericardium: Normal pericardium. IV bubble contrast : A right-left intra atrial shunt is detected that appears to be coming from the left pulmonary veins

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24 Unspecified ILD, too risky to biopsy but could be sarcoidosis Severe PH with elevated L sided pressures Started on inh. Treprostinil after extensive discussion with patient Clinically improved but remained hypoxic Died suddenly at home after about 12 months of stability

25 Case 3 Massive acute PE on a background of chronic PE 1/2013. Course complicated by retroperitoneal bleed after tpa x2. Hemodynamics 3/2013 revealed severe disease with mean PAP 55mmHg RAP 15 CO/Cl 4.1 and 2.1 PVR Initially treated with high dosed sildenafil and repeat RHC 12/2013 showed progression. PTE on 4/8/2015. Despite large amount of chronic clot removal, IVC filter placed he had evidence of significant residual pulmonary HTN.

26 Post-op day 4 RAP 16 PAP 66/33 (42) CVP used for PAOP CO/CI 4.8/2.5 PVR 5.5

27 Started on Adempas since did not tolerate Sildenafil Echo mentions: Severe RA and RV dilation with pressure volume overload and a 2.3 x 1.5 cm mass that up takes contrast so likely not thrombus??? Est PASP 90 Continued on Adempas

28 Moves to Jacksonville and admitted via ER on the day of clinic evaluation for SOB

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32 R Heart Cath Hemodynamics: HR: 99 MAP: 90mmHg RA: 24mmHg RV systolic: 63mmHg RV diastolic: 3mmHg PA systolic: 91mmHg PA diastolic: 51mmHg PA mean: 67mmHg PCWP: 7mmHg BSA: 1.89 TPG (PAD-PAWP): 44 CO (thermodilution): 3.21L/min CI (thermodilution): 1.69L/min/m2 PVR (thermodilution): 1495dynes.sec/cm5 SVR (thermodilution): 1644dynes.sec/cm5 CO (Fick's): 3.26L/min CI (Fick's): 1.73L/min/m2 PVR (Fick's): 1472dynes.sec/cm5 SVR (Fick's): 1619dynes.sec/cm5 Chamber sats: Arterial: 93.6% Low SVC: 44.9% PA: 46.6%

33 At 22 ng/kg/min HR: 125 MAP: 93mmHg RA: 13mmHg PA systolic: 121mmHg PA diastolic: 42mmHg PA mean: 70mmHg PCWP: 9mmHg BSA: 1.73 TPG (PAD-PAWP): 33 CO (Fick's): 4.73L/min CI (Fick's): 2.75L/min/m2 PVR (Fick's): 1031dynes.sec/cm5 SVR (Fick's): 1353dynes.sec/cm5 Chamber sats: Arterial: 93.9% Low SVC: 77.0% PA: 63.2% CO (thermodilution): 5.21L/min CI (thermodilution): 2.75L/min/m2 PVR (thermodilution): 936dynes.sec/cm5 SVR (thermodilution): 1228dynes.sec/cm5

34 Severe residual PH? J Thorac Cardiovasc Surg Feb;141(2): doi: /j.jtcvs Epub 2010 May mo after operation Group 1 (n = 210) Group 2 (n = 96) P value mpap (mm Hg) 20 ± 5 38 ± 8 <.001 PVR (dynes s 1 cm 5 ) 181 ± ± 250 <.001 CI (L min 1 m 2 ) 2.5 ± ± 0.62 NS SMWD (m) 386 ± ± 97 <.001 NYHA class I or II (n) 88.1% (170/193) 68.9% (62/90) <.001 Table 2. Comparison between patients without (group 1) or with (group 2) residual pulmonary hypertension (median ± interquartile range) mpap, Mean pulmonary artery pressure; PVR, pulmonary vascular resistance; CI, cardiac index; NS, not significant; SMWD, 6-minute walk distance; NYHA, New York Heart Association.

35 Case 4 28 African woman presented with SOB Echo done at an ER showed severe Pulm HTN was started on Bosentan by outside physician

36 RAP 35 PAP 160/75 PA mean 103 PCWP 20 C.O Fick 3 L/min, C.I fick 1.7 L/min/m2 PVR 2213

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40 Started on IV treprostinil and titrated up to 50 while inpatient Despite aggressive diuresis unable to decrease RAP

41 R heart cath RAP 35 PAS 159 mm of Hg PAD 75 mm of Hg PA mean 103 mm of Hg PAOP 18 mm of Hg C.O 5.63 C.I 3.47 PVR 1193 MAP 75 HR 103

42 Returned due to nausea vomiting and rectal bleeding Transitioned from IV remodulin to IV Epo Today on 40ng/kg/min of epo and 32 ng/kg/min of treprostinil during the transition from Treprostinil to epo hemodynamics show: RAP 30, PAP 140/60 PA mean 87, PCWP 17, C.O (TD) 5.56 L/min, C.I (TD) 3.25 L/min/m2, PVR Final epos dose was 110 ng/kg min Developed AKI

43 Severe Nausea, vomiting, started CRRT, CVP remained high, hypotensive required pressors, unable to decrease RAP through out the course, eventually multiple pressors and had a cardiac arrest and died.

44 Case 5 46 woman diagnosed with pulmonary arterial hypertension in July 2012 after a R and L heart cath: RAP mean 5 PA mean 48 PCWP 8 C.I/C.O 2.1/3.8 PVR about 10 WU She was started on Revatio and subsequently Tracleer, Currently on Revatio 20 mg TID and Tracleer 125 mg BID She has noticed a decline in her clinical symptoms of exertional SOB and currently is WHO class 3 on Tracleer and Revatio A repeat RHC done May showed RAP mean 8 PA mean 52 PCWP 13 C.I/C.O 2.3/4.5 PVR about 8 WU

45 Echo shows evidence of RV dilation and RV pressure volume overload and R to L shunt via PFO She also has a pulmonary AVM in LUL by CTA done twice She is currently anti coagulated with warfarin and is on oxygen also. She has symptoms suggestive of syncope with exertion.

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47 Case 5 46 woman diagnosed with pulmonary arterial hypertension in July 2012 after a R and L heart cath: RAP mean 5 PA mean 48 PCWP 8 C.I/C.O 2.1/3.8 PVR about 10 WU She was started on Revatio and subsequently Tracleer, Currently on Revatio 20 mg TID and Tracleer 125 mg BID She has noticed a decline in her clinical symptoms of exertional SOB and currently is WHO class 3 on Tracleer and Revatio A repeat RHC done May showed RAP mean 8 PA mean 52 PCWP 13 C.I/C.O 2.3/4.5 PVR about 8 WU

48 Echo shows evidence of RV dilation and RV pressure volume overload and R to L shunt via PFO She also has a pulmonary AVM in LUL by CTA done twice She is currently anti coagulated with warfarin and is on oxygen also. She has symptoms suggestive of syncope with exertion.

49 Admitted with the aim of embolization of AVM while on IV epo

50 PAP before and after temporary occlusion of the large AVM 79/19 (46 ) 76/20 (42 )

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52 Post embolization kept anticoagulated but symptoms progressed requiring escalating doses of epo IV and currently on 94 ng/kg/min along with Macitentan 10 mg and Revatio 20 mg TID