Pulmonary Hypertension Surgical Options. Primary pulmonary hypertension. Transplantation. Thromboembolic disease Endarterectomy

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1 Stuart W. Jamieson Cardiothoracic Surgery University of California San Diego Chronic Thromboembolic Pulmonary Hypertension Pulmonary Hypertension Surgical Options Primary pulmonary hypertension Transplantation Thromboembolic disease Endarterectomy Single lung Double lung Heart - lung Embolic pulmonary hypertension: Incidence & Prevalence What is the incidence of pulmonary hypertension due to thromboembolic disease? Embolic pulmonary hypertension: Incidence & Prevalence More prevalent than recognized USA: Estimated 63, acute symptomatic PE s with 5, survivors (1983) About 5% risk of chronic pulmonary hypertension following an acute PE Estimated 25, cases annually (USA) Embolic pulmonary hypertension: Incidence & Prevalence More prevalent than recognized USA: Estimated 63, acute symptomatic PE s with 75, survivors (211) About 5% risk of chronic pulmonary hypertension following an acute PE Estimated 35, cases annually (USA) Other causes Indwelling catheters, leads, coagulation abnormalities, secondary vasculopathy? Number of cases of asymptomatic PE? >5, yearly in USA Embolic pulmonary hypertension: Incidence & Prevalence Under recognized condition 1

2 Treatment Medical: Limited and temporary Cannot remove mechanical obstruction Only supportive, does not affect the prognosis Symptoms generally not improved, unless obstruction is relieved Surgical: Mainstay of treatment Pulmonary endarterectomy Transplantation inappropriate Pulmonary Endarterectomy Important Concepts No disease is too distal for surgery (no such thing as surgically inaccessible ) No degree of pulmonary hypertension, PVR or RV failure too severe for operation Pulmonary Angiography - combined with right heart catheterization The key to Diagnosis PTE Specimen - Most valuable is to match PVR with extent of disease seen on pulmonary angiogram. - PVR / angiographic discordance will predict likely result does not contraindicate operation 2

3 Pulmonary angiogram Pulmonary angiogram PVR pre-op >1,2 PVR post-op < 3 Pulmonary angiogram Pulmonary angiogram 3

4 Operation Tends to be underestimated Significant learning curve Bilateral Mandatory Circulatory arrest The Surgery Median sternotomy Cardiopulmonary bypass Deep hypothermic circulatory arrest (DHCA) Both pulmonary arteries are treated Complete endarterectomy ASD repair and other procedures RV RA The head is wrapped in a circulating cold water cooling blanket in preparation for Deep Hypothermic Circulatory Arrest (DHCA) 4

5 Assessment of the cooling jacket in 55 patients - Rectal temperature o C Bladder temperature o C Tympanic temperature o C ** ** p <.1 Removing the specimen Classification of Operative Specimens: TYPE I: 15-2% of cases, major vessel clot is present and readily visible upon opening of PA TYPE II: 6% of cases, no major vessel thrombus, thickened intima with webs TYPE III: 15-2%, very distal disease, confined to segmental and sub-segmental branches TYPE IV: No thromboembolic pulmonary hypertension, inoperable, intrinsic small vessel disease. Type 1 Type 1 Type 1 Type I Type 2 5

6 Type 1 fresh clot Type 1 old clot Type 1 Type I Type 2 Type 1 Type 1 Type 1 - Calcification Type I Type 2 Type I Type 2 6

7 Type 1 - Calcification Type 2 Type 2 Segmental disease Type 3 Segmental disease Type 3 Type 3 7

8 Primary pulmonary hypertension (Type IV) PPH - no distal occlusion.. Results >2,6 cases at UCSD Medical Center Trousers, not tails Mean age 51 (ranging from 8 to 88) Slight male predominance One third had at least one additional cardiac procedure (PFO, ASD, CABG, etc.) Average operative procedure was 7 hours Cardiopulmonary bypass min Myocardial ischemia time min Circulatory arrest time min Results <18 yrs. Results <18 yrs. Results 17 patients < 18 years Range 8 to 18 Operated on between % had underlying hypercoagulable state Lupus anticoagulant (5) Anticardiolipin antibody (4) Protein C deficiency (3) NOT seen: Antithrombin III, Factor V Leiden, Prothrombin gene defects 17 patients < 18 years Range 8 to 18 Operated on between INITIAL DIAGNOSIS: PPH 5 Atypical pneumonia 3 Asthma 2 1. Hemodynamic 2. Mortality 3. Long term 8

9 Results 1. Hemodynamic 2. Mortality 3. Long term Pre & Post-op hemodynamics Sys PA (mm Hg) PVR (dynes/sec/cm-5) Pre-op Post-op 3.84 Mean PA (mm Hg) C.O. (L/min) Pre & Post-op hemodynamics - < 18 years Sys PA (mm Hg) PVR (dynes/sec/cm-5) Pre-op Post-op 3.8 Mean PA (mm Hg) C.O. (L/min) Results 1. Hemodynamic Overall Mortality By Era Hemodynamic Complete resolution anticipated in >9% of cases 2. Mortality 3. Long term If pre-op PVR > 1, complete resolution anticipated if PVR concordant with angiographic appearances 9

10 Mortality By Pre-op PVR Mortality Last Two Years Mortality < 18 years old Total Number: 27 patients No Mortality for Isolated PTE Excluding Combined Procedures PTE/CABG PTE/Valve PTE/Valve/CABG Excluding Sarcoma Patients Total Number: 17 patients No operative mortality 2 late deaths: rethrombosis (1) residual pulmonary hypertension (PVR 3,72 to 1,311) Survival at 5 years = 87.5% Results 1. Hemodynamic 2. Mortality 3. Long term Encouraging. Long-term Outcome Long-term anticoagulation, IVC filter Most patients return to NYHA class I or II Improved autoregulation in pulmonary vascular bed Remodeling of right (thus left) side of the heart, with improved function Archibald et. Al.: Long term outcome after pulmonary thromboendarterectomy. Am J Resp Crit Care Med 1999; 16: Pulmonary Endarterectomy (versus transplantation) Low mortality rate Elective surgery Considered a cure Coumadin only 1

11 Pulmonary Endarterectomy Transplantation NEVER appropriate for thromboembolic pulmonary hypertension EVERY patient whose etiology is thromboembolic is a surgical candidate Patient at 12, feet Conclusion: Condition Under-recognized Patient at 12, feet Conclusion: Poor prognosis Patient at 12, feet 11

12 Conclusion: Ineffective medical therapy Patient at 12, feet Conclusion: Surgical therapy curative Patient at 12, feet Conclusion: Operation requires circulatory arrest, absolutely bloodless field, and careful cooling and rewarming techniques Thank you UCSD Cardiovascular Center 12