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CPC -5
Clinical Discussion
Steven R. Jones, MD
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
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?
Imaging
Chest CT and MRI 2003, 2004 Calcification of PA Calcification of Ao Mixed AS/AR with sclerotic AoV Moderate MR Pericardium normal
Timeline of Illness
HLChest XRT
Dyslipidemia
Childhood
SOB:SVG-RCAEarly AS
2000 20042003 2007
DEATH
SOB3VD/LM
Mod AS/AR
CABAVR
Sx improved
Heart,Vessels,
Pericardium1st Hit
Pericardium,Myocardium
2nd Hit
Extravascular sclerosis,Atherosclerosis
CurrentJHH
Admission
Extravascular sclerosis,Atherosclerosis
Lipids, diet, risk factors, time
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
Clinical Diagnoses - 2007 Admission
2. Mitral Regurgitation
3. Pulmonary hypertension Post capillary - 2o 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 2o to hepatic congestion
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
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/m2
Est. SVI 25 mL/m2 (normal 40-50 mL/m2)
High RVSP and diastolic pressure near equalizationconsistent withrestrictive CM +pericardial constriction
Restriction vs. ConstrictionRestrictive Cardiomyopathy
Adapted from Benotti et al. Circulation 1980; 61: 1206.
Near, but not exact trackingof LV, RV diastolic pressurewith LA, RA.
Absent Kussmaul’s sign.
Restriction vs. ConstrictionPseudo-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.
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
Hospital Course
Clinical improvement, ambulatory
Sudden death - PEA
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
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
Final Diagnosis—Cause of Death
PEA resulting from radiation induced restrictive cardiomyopathy,
RV/LV failure.