10th Block: Cardiovascular

COR PULMONALE

dr. Romi Ermawan, Sp.JP FIHA

Cardiology and Vascular Medicine
Medical Faculty of Mataram University
 DEFINITION

A situation in which there is

hypertrophy and dilatation of the right
ventricle
As a result of pulmonary arterial
hypertension
Caused by intrinsic disease of the lung
parenchyma, pulmonary vascular and
thoracic wall abnormality
Without Mitral Stenosis, Congenital
Heart Disease or Left Heart Failure
 PHYSIOLOGY

SVC
Ao 120/80

LA
10
RA
5
LV
"WEDGE" 120/0-10
25/0-5 12
RV

IVC

"WEDGE”
12 ~ PCWP ~ V.P ~ L,A ~ 10
PATHOPHYSIOLOGY

Lung disease
• Structurally
• Functionally

Right Heart Abnormality

• RV dilatation
• RV hypertrophy

RIGHT HEART FAILURE

ETIOLOGY

Chronic Obstructive
Pulmonary Disease
(COPD)

Chronic Hypoventilation

Abnormalities in
Pulmonary Vasculature
ETIOLOGY
Chronic Obstructive
Pulmonary Disease
(COPD)

• Chronic bronchitis
• Emphisematous Lung
• Chronic bronchial asthma
• Lung TB (destroyed lung)
• Pneumoconiosis
• etc..
ETIOLOGY

Chronic Hypoventilation
• Thorax deformities
• Kyphoscoliosis
• Obesity Hypoventilation
Syndrome (Pickwickian
Syndrome
• etc..
ETIOLOGY
Abnormalities in
Pulmonary Vasculature
• Embolism
• Thrombosis
• Primary Pulmonary HT
• Diffuse vasculitis
 BRONCHO-PULMONARY DISEASE

BRONCH BRONCH BR. MOTOR

SPASM OBSTRUCTION TONE ↑

VENTILATION ↓ ALVEOLAR PRESS ↑ VASCULER BED. ↓

HYPOXEMIA PULM. VASOCONSTRICTION PVR ↑

POLICITEMIA PULMONARY HT ATHEROSCLEROSIS

RVH

COR PULMONALE

COMPENSATED DECOMPENSATED
 DIAGNOSIS

History Taking Shortness of breath

Cough

Underlying lung disease

Physical Finding Dyspnea

Cyanosis

Elevated JVP

Barrel chest

Emphisema

Rhales

Wheezing
 DIAGNOSIS

ECG Right Axis Deviation

Arrhythmias

RV Hypertrophy

Right Atrial Abnormality

Chest X Ray Lung disease

RV Hypertrophy

Laboratory Policitemia

Hypercarbia

Hypoxemia
 MANAGEMENT

LUNG DISEASE

PULMONARY HYPERTENSION

RIGHT HEART FAILURE

MANAGEMENT FOR PULMONARY HYPERTENSION

Main goals:
Relieving the symptoms
Improving the quality of life
Increasing the survival

1. Chronic hypoxia: supplemental O2 with a target saturation ≥ 90%

2. Right heart failure: diuretics & digoxin (if AF)
3. Responsive to acute vasodilator testing: long term CCB therapy (if
bradycardia use nifedipine or amlodipine, if tachycardia use diltiazem)
MANAGEMENT FOR PULMONARY HYPERTENSION
SPECIFIC PULMONARY ARTERY VASODILATORS
MANAGEMENT FOR PULMONARY HYPERTENSION
SPECIFIC PULMONARY ARTERY VASODILATORS
MANAGEMENT FOR PULMONARY HYPERTENSION
SPECIFIC PULMONARY ARTERY VASODILATORS
Prostacyclin Derivates

Continuous (iv) Epoprostenol

• Anti-proliferative and anti-vascular-remodeling
• Started at 2 ng/kg/min, titrated up to maximum tolerated dose
• After 1 year maintain at 50-80 ng/kg/min
• As a standard therapy in unresponsive patients to acute
vasodilator testing, or who experienced a clinical worsening
after CCB therapy

Nebulized Iloprost
• Duration of action for 60 minutes, can be repeated if
necessary
• 2.5-5 mg, can be administered 6-9 x/day
• Not recommended in unstable condition or severe right heart
failure
Ford. Arch Dis Child Educ Pr Ed. 2005;90:ep15–ep20.
MANAGEMENT FOR PULMONARY HYPERTENSION
SPECIFIC PULMONARY ARTERY VASODILATORS
Prostacyclin Derivates

Continuous (sc) Treprostinil

Started at 1.25 ng/kg/min, titrated up to maximum tolerated
dose

Oral Beraprost
• Hasn’t been studied widely in children
• Adult dose: started at 20 µg 4 x/day, titrated up to 480 µg daily

Ford. Arch Dis Child Educ Pr Ed. 2005;90:ep15–ep20.

MANAGEMENT FOR PULMONARY HYPERTENSION
SPECIFIC PULMONARY ARTERY VASODILATORS
Endothelin Receptor Antagonist
Oral Bosentan
• A competitive receptor antagonist of ET-A and ET-B
• Already registered in European Medicines Pediatrics Agency
• According to body weight:
‣ 10-20 kg: 31.25 mg/day for 4 weeks, increased to 2 x 32.5 mg/day
‣ 20-40 kg: 31.25 mg 2x/day for 4 weeks, increased to 2 x 62.5 mg/day
‣ > 40 kg: 62.5 mg 2x/day for 4 weeks, increased to 2 x 125 mg/day

Phosphodiesterase Inhibitor

Oral Sildenafil
Currently the RCT on efficacy and dosing in children are still being held

Ford. Arch Dis Child Educ Pr Ed. 2005;90:ep15–ep20.

Galie` et al. Eur Heart J. 2009;30:2493–537.
MANAGEMENT FOR RIGHT HEART FAILURE

• Bed rest

• Low salt diet

• Proper fluid balance

• Diuretics (furosemide and spironolactone)

• Digitalis (digoxin)
 PROGNOSIS

• Cor pulmonale due to COPD had 5 years of survival

rate of 30%

• The media survival was 2.8 years, with 1 year; 3 years;

5 years survival rate of 65%; 50%; 30%
?