CPC -5 Clinical Discussion

1
CPC -5Clinical 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
Heart, Vessels, Pericardium 1st Hit
Pericardium, Myocardium 2nd Hit
Extravascular sclerosis, Atherosclerosis
Extravascular sclerosis, Atherosclerosis
D E A T H
HL Chest XRT Dyslipidemia
SOB SVG-RCA Early AS
SOB 3VD/LM Mod AS/AR
CAB AVR Sx improved
Current JHH Admission
2000
2004
2003
2007
Childhood
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
• RVgtLV myocardial fibrosis, failure
• Pericardial fibrosis, ?constriction

7
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

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/m2
• Est. SVI 25 mL/m2 (normal 40-50
  mL/m2)

High RVSP and diastolic pressure near
equalization consistent with restrictive CM
pericardial constriction
10
Restriction vs. Constriction
Restrictive Cardiomyopathy
Near, but not exact tracking of LV, RV diastolic
pressure with LA, RA. Absent Kussmauls sign.
Adapted from Benotti et al. Circulation 1980
61 1206.
11
Restriction vs. Constriction
Pseudo-constrictive physiology of acute severe MR
Can result from any acute or subacute volume
load even with normal pericardium.
Adapted from Bartle et al. Circulation 1967 36
839.
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 DiagnosisCause of Death

• PEA resulting from radiation induced restrictive
  cardiomyopathy,
• RV/LV failure.