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.