Clinical History. CHD-PAH Case: Physical Exam. Clinical History. To Repair or not to Repair?

Transcription

1 CHD-PAH Case: To Repair or not to Repair? Erika Berman Rosenzweig, MD Columbia University, College of Physicians and Surgeons Clinical History 18 year old female had been previously well, but new murmur was noted by PMD Referred to a cardiologist for evaluation which included an echocardiogram Echocardiogram revealed a large secundum ASD and elevated estimated RVSP Clinical History Referred to Columbia University Pulmonary Hypertension Center for further evaluation Admits to tiring sooner than peers on walks (SOB), but otherwise denies all cardiorespiratory symptoms (WHO II) No known risk factors for PH (altitude, diet pills, OCP, HIV, PE, DVT, negative family history: CTD, CHD, PH) Physical Exam Well appearing, very thin female in no distress Vitals: BP 100/70mmHg, P 80, RR 16, resting RA saturation 97%, 37kg Chest: Clear to auscultation, no rales Cor: RR nl S1 with prominent/palp S2 and III/VI pansystolic murmur at LLSB Abd: No HSM, no ascites Ext: No clubbing, cyanosis or edema 1

2 ECG Chest Xray Echocardiogram: Baseline 2

3 Baseline Labs Hgb 12.6gm/dl ANA negative BNP 698 Uric Acid 5.4mg/dl Hgb AA HIV negative Hypercoagulable w/u negative Management Options Cardiac catheterization Acute vasodilator testing Close ASD Interventional vs. Surgical Partial vs. complete No Intervention Medical intervention With or without intent to close in future Management Options: Further Evaluation Cardiac Catheterization Acute vasodilator testing Baseline Exercise Testing 6 minute walk: 600 meters Oxygen saturation 96% at rest, 92% with exertion CPET: Peak workload: 44 watts (42% predicted) Peak VO2: 15.9 ml/kg/min (43% predicted) VE/VCO2: 53 ETCO2: 25 3

4 Cardiac Catheterization: Baseline Resting Room Air RAPm 4 PCWPm 6 PAP 92/31/51 SAP 94/50/68 PVR 10.9 U*m2 RP:RS 0.4 CI 2.3 QP:QS 1.8:1 Acute Vasodilator Testing: Baseline vs. AVT Resting (RA) AVT(iNO) RAPm 4 4 PCWPm 6 7 PAP 92/31/51 94/28/49 SAP 94/50/68 99/54/72 PVR 10.9 U*m2 8.9 U*m2 RP:RS CI QP:QS 1.8:1 2.5:1 Temporary Balloon Occlusion: Baseline vs. Balloon Occlusion RESTING ROOM AIR RAPm 4 PCWPm 6 PAP 92/31/51 SAP 94/50/68 PVR 10.9 RP:RS 0.4 CI 2.3 QP:QS 1.8:1 BALLOON OCCLUSION RAP 4 PCWP 13 PAP 93/28/49 SAP 104/49/70 PVR 12.9 RP:RS 0.4 CI 2.0 QP:QS 1.1:1 Management Options Cardiac Catheterization Acute Vasodilator Testing Close ASD Interventional vs. Surgical Partial vs. complete No intervention Medical intervention With or without intent to close in future 4

5 What are the current criteria to close CHD (ASD) in patients with pulmonary hypertension? Previous use of lung biopsy grades to determine operability; high risk Are there resting hemodynamic criteria? Acute vasodilator testing: Is the definition used for IPAH patients appropriate for CHD pts where resistance is the issue? What is the role of temporary balloon occlusion? Risks vs. Benefits of Repair of CHD-PH patient Benefit Abort Right-to-Left shunting Decrease cerebrovascular events (stroke/abscess) Prevent cyanosis Improve exercise capacity Decrease erythrocytosis Decrease hemostatic probs Decrease systemic organ failure Protect Pulmonary Circulation Potential Risk Potential conversion of Eisenmenger physiology to IPAH physiology High perioperative risk Very limited experience and no long-term data available Konstantinos D, et al. Int J of Card, 2008 Management: Complete Surgical Closure of ASD with PAH Steele PM, et al reported outcome at 4 yrs of 40 pts with ASD and TPR > 7 W*u ( ) 26 underwent surgery, 14 medically treated Median follow up was 12 yrs 17/40 pts died at most recent follow up Predictors of Poor Outcome for surgically treated patients: TPR > 15 Woods units (p<0.001) Saturation <92% at diagnostic catheterization for surgically treated pts was also predictive of death (NS) Management Options Cardiac Catheterization Acute Vasodilator Testing Close ASD Interventional vs. Surgical Partial vs. complete No intervention Medical intervention With or without intent to close in future TPR = total pulmonary resistance =PAPm/pulmonary index (QP) Steele PM, et al, Circulation,

6 Management: No Intervention Eisenmenger Syndrome Natural History Management Options Cardiac catheterization Acute vasodilator testing Close ASD Interventional vs. Surgical Partial vs. complete No Intervention Medical intervention (medical or palliative surgical) With or without intent to close in future Diller, et al. Circulation 2007 Is There a Role for Pre-treatment for CHD-PH (ASD) Which agent (s)? How long? Role of other risk factors, e.g. age, associated conditions, family history,etc. Considerations (short-term benefit for questionable long-term effect on vascular bed) Is there a role for staged surgical intervention for other lesions, e.g. VSD PA banding (VSD) Medical Treatment: Post-operative Which agents? Follow up assessment (how often) Long-term outcome unknown 6

7 CHD/PH (ASD) Clinical Management Algorithm: Individualized Case Approach Resting PVR < 3 RP:RS ratio < 0.33 TBO no fall in CI Exercise Testing Desaturation with exercise No Desaturation with exercise Cardiac Catheterization AVT: PVR >3 PVR:SVR >0.33 Resting PVR > 3 RP:RS ratio >0.33 +/-TBO Treat Medically Serial Re-assessments (Cath/Exercise) AVT: PVR<3 Or PVR:SVR <0.33 TBO: no fall in CO No desaturation with exercise Consider partial closure +/- medical treatment Clinical Case: Management Follow up Patient treated with an ERA 1 year follow up Catheterization (no significant change); ERA changed to alternate ERA 2 year follow up: Clinically asymptomatic WHO class I Catheterization: baseline vs. medical tx TBO=temporary balloon occlusion Close ASD If resting PVR <3 Or RP:RS ratio falls below 0.33 Consider partial closure +/_medical treatment Follow Up Catheterization: Baseline vs. Medical tx Resting (RA) Medical Tx RAPm 4 7 PCWPm 6 6 PAP 92/31/51 84/34/51 SAP 94/50/68 92/51/64 PVR 10.9 U*m2 6.8 U*m2 RP:RS CI QP:QS 1.8:1 3.0:1 Case: Management Patient taken to OR for fenestrated (4mm) closure of a large secundum ASD (6/07) Postoperative course relatively uncomplicated Follow up catheterization 5 mos post op (PAP 65/28/41, SAP, 90/52/64, CI 2.4, PVR 10.7, RP:RS 0.4, QP:QS 1.3:1) PDE-5 inhibitor added 20 mg po tid 7

8 Post-operative Echocardiogram Post op ECHO Late Post op data (12/08) WHO functionai class I Labs: BNP 261 (698 baseline) 6 minute walk distance 612 meters (baseline 600); saturation 96%>95%. CPET: Watts 49 (46%) Baseline (42%) VO2/kg 14.5 (41%) (43%) ETCO2 40 (53) VEVCO2 34 (25) Follow Up Catheterization: Baseline vs. Med tx vs. Post-op 1yr Baseline Medical Tx Post-op/Recent RAPm PCWPm PAP 92/31/51 84/34/51 62/27/40 SAP 94/50/68 92/51/64 125/72/90 PVR RP:RS CI QP:QS 1.8:1 3.0:1 1.3:1 8

9 Case: Conclusion 18 year old with large secundum ASD and pulmonary hypertension. Deemed inoperable at initial evaluation Pre-treated with ERA Underwent partial surgical repair of ASD Post-treated with ERA and PDE-5 inhibitor Clinically well; improved hemodynamics?long-term outcome? Did we alter natural history? Unknown Take Home Message Management of patients with CHD-PH is complex, with optimal standardized approach still unknown. With novel agents, may be possible to include medical management in the treatment approach, both pre and post-operatively. However, one must be certain before surgical intervention not to convert a patient with Eisenmenger physiology into a patient with IPAH for whom the natural history is worse. Serial reassessment is required. 9