1. CPC -5 Clinical Discussion Steven R. Jones, MD

2. Central Features of History HL - chest radiotherapy Premature CAD dysplipidemia, otherwise limited CV risk 1VD RCA, initial dx 2000 at age 43 Rapid progression to 3VD/LM CAD, CAB 2004 Valvular heart disease Sclerotic AoV leading to AVR at 2004 surgery Severe MV calcification, MR

3. Central Features of History Hemodynamic presentations Fluid retention, edema Exercise intolerance, fatigue Dyspnea No history of angina Was CAD ever responsible for symptoms? Prognostically important, but incidental?

4. Imaging Chest CT and MRI 2003, 2004 Calcification of PA Calcification of Ao Mixed AS/AR with sclerotic AoV Moderate MR Pericardium normal

5. Timeline of Illness HL Chest XRT Dyslipidemia Childhood SOB: SVG-RCA Early AS 2000200420032007 DEATHDEATH SOB 3VD/LM Mod AS/AR CAB AVR Sx improved Heart, Vessels, Pericardium 1 st Hit Pericardium, Myocardium 2 nd Hit Extravascular sclerosis, Atherosclerosis Current JHH Admission Extravascular sclerosis, Atherosclerosis Lipids, diet, risk factors, time

6. Clinical Diagnoses - 2007 Admission 1. Radiation injury leading to: CAD, accelerated by dyslipidemia, gout, obesity Valvular sclerosis with resulting AR/AS, MR, PR Calcification of great vessels RV>LV myocardial fibrosis, failure Pericardial fibrosis, ?constriction

7. Clinical Diagnoses - 2007 Admission 2. Mitral Regurgitation 3. Pulmonary hypertension Post capillary - 2 o to MR and increased LA pressure 4. Suspected restriction/pericardial constriction- Complicated by MR and RV/LA volume loading 5. Edema, high CVP 6. Increased INR 2 o to hepatic congestion

8. Hospital Course Poor response to diuretics, rising creatinine Compromised SV, CO, perfusion pressure  Restriction/Pericardial constriction  Severe MR  Failing RV/LV Need to sustain RV, LV preload Cardiorenal syndrome

9. Hospital Course Right Heart Catheterization RA mean 27 mmHg RV 67/29 mmHg PA 67/31 mmHg PCWP mean 31 mmHg BP 95/70 mmHg CI 2.4 L/min/m 2 Est. SVI 25 mL/m 2 (normal 40-50 mL/m 2 ) High RVSP and diastolic pressure near equalization consistent with restrictive CM + pericardial constriction

10. Restriction vs. Constriction Restrictive Cardiomyopathy Adapted from Benotti et al. Circulation 1980; 61: 1206. Near, but not exact tracking of LV, RV diastolic pressure with LA, RA. Absent Kussmaul’s sign.

11. Restriction vs. Constriction Pseudo-constrictive physiology of acute severe MR Adapted from Bartle et al. Circulation 1967; 36: 839. Can result from any acute or subacute volume load even with normal pericardium.

12. Hospital Course Improved response with Milrinone Inotropic support of failing RV Pulmonary vasodilator  reduced PA pressure  Improved pulmonary congestive symptoms Peripheral vasodilator  reduced MV regurgitant load, regurgitant fraction  increased forward SV Preservation of renal perfusion in face of diuresis

13. Hospital Course Clinical improvement, ambulatory Sudden death - PEA

14. Cause of Death Pulmonary embolism  PEA  High CVP, edema, sluggish flow in dilated veins  Prolonged bed rest, hospitalization CAD  Acute myocardial infarction  Primary or secondary arrhythmias usually VT/VF

15. Cause of Death SCD in setting of heart failure  Radiation injury heart – fibrosis, failure  High catecholamine levels HR ~90-100  Inotropic support  Intracellular Ca ++ overload  Contraction band necrosis  Typical rhythm leading to death: asystole or PEA

16. Final Diagnosis—Cause of Death PEA resulting from radiation induced restrictive cardiomyopathy, RV/LV failure.