Cor pulmonale ALOK SINHA Department of Medicine Manipal

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Cor pulmonale ALOK SINHA Department of Medicine Manipal College of Medical Sciences Pokhara, Nepal

Cor pulmonale ALOK SINHA Department of Medicine Manipal College of Medical Sciences Pokhara, Nepal

Cor pulmonale is defined as an alteration in the structure and function of the

Cor pulmonale is defined as an alteration in the structure and function of the right ventricle caused by a primary disorder of the respiratory system – lung parenchyma, lung vasculature or thoracic cage. Right sided heart disease secondary to lung disease Pulmonary hypertension is the common link between lung dysfunction and the heart in cor pulmonale

Pathophysiology 1. Pulmonary vasoconstriction due to a. Hypoxia b. Blood acidemia 2. Obliteration of

Pathophysiology 1. Pulmonary vasoconstriction due to a. Hypoxia b. Blood acidemia 2. Obliteration of the pulmonary vascular bed secondary to lung disorders – a. emphysema b. pulmonary thrombo embolism c. interstitial lung disease

3. increased blood viscosity secondary to blood disorders l polycythemia vera l sickle cell

3. increased blood viscosity secondary to blood disorders l polycythemia vera l sickle cell disease l macroglobulinemia 4. idiopathic primary pulmonary hypertension

Pul art pressure – dilatation of R V – reduced C O & septal

Pul art pressure – dilatation of R V – reduced C O & septal displacement-decrease L. V. volume – decresed coronary blood to R V – further detoriation of R V function Septum pushed to left Reversed Bernmeim’s effect

patient with acute pulmonary hypertension due to pulmonary embolism After clot lysis

patient with acute pulmonary hypertension due to pulmonary embolism After clot lysis

Acute cor pulmonale a. massive pulmonary embolism (more common) b. acute respiratory distress syndrome

Acute cor pulmonale a. massive pulmonary embolism (more common) b. acute respiratory distress syndrome (ARDS). is associated with R V dilatation

l. Chronic cor pulmonale l C O P D > 50% of cases

l. Chronic cor pulmonale l C O P D > 50% of cases

Disorders with primary involvement of pulmonary vasculature and circulation l. Repeated pulmonary emboli l.

Disorders with primary involvement of pulmonary vasculature and circulation l. Repeated pulmonary emboli l. Pulmonary vasculitis l. Pulmonary veno-occlusive disease l. Sickle cell disease l. High altitude disease with pulmonary vasoconstriction l. Primary pulmonary hypertension

Disorders with secondary involvement of pulmonary vasculature and circulation l Parenchymal lung diseases l.

Disorders with secondary involvement of pulmonary vasculature and circulation l Parenchymal lung diseases l. Chronic obstructive l interstitial lung diseases l Neuromuscular pulmonary diseases disorders l myasthenia gravis l Poliomyelitis l amyotrophic lateral sclerosis l Obstructive and central sleep apnea l Thoracic deformities l Kyphoscoliosis l Ankylosing spondylitis

CLINICAL FEATURES

CLINICAL FEATURES

Clinical manifestations of cor pulmonale nonspecific symptoms subtle in early stages of the disease

Clinical manifestations of cor pulmonale nonspecific symptoms subtle in early stages of the disease mistakenly attributed to underlying pulmonary pathology which are: l. Easy fatigability l Tachypnea l Exertional dyspnea l Cough Followed by

1. Anginal chest pain l Right ventricular ischemia (does not respond to nitrates) l

1. Anginal chest pain l Right ventricular ischemia (does not respond to nitrates) l Rt. coronary artery stretching in dilated A-V groove following RVH 2. Hemoptysis because of rupture of a dilated or atherosclerotic pulmonary artery

3. A variety of neurologic symptoms may be seen due to decreased cardiac output

3. A variety of neurologic symptoms may be seen due to decreased cardiac output and hypoxemia l impaired cognitive & higher mental functions

4. Rarely hoarseness due to compression of the left recurrent laryngeal nerve by a

4. Rarely hoarseness due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery 5. In advanced stages, passive hepatic congestion secondary to severe right ventricular failure lead to l anorexia l right upper quadrant abdominal discomfort l jaundice

6. Syncope with exertion lseen in severe disease lreflects a relative inability to increase

6. Syncope with exertion lseen in severe disease lreflects a relative inability to increase cardiac output during exercise with a subsequent drop in the systemic arterial pressure 7. Peripheral edema

Physical findings

Physical findings

may reflect a. The underlying lung disease b. pulmonary hypertension c. RVH d. RV

may reflect a. The underlying lung disease b. pulmonary hypertension c. RVH d. RV failure

On inspection 1. An increase in chest diameter 2. Laboured respiratory efforts with retractions

On inspection 1. An increase in chest diameter 2. Laboured respiratory efforts with retractions of chest wall 3. distended neck veins with prominent “a” or giant “v” waves 4. cyanosis may be seen

l RVH - characterized by l Epigastric pulsation l left parasternal heave l Apex

l RVH - characterized by l Epigastric pulsation l left parasternal heave l Apex beat: in 5 th ICS outside MCL diffuse, ill suatained l + Hepatojugular reflex and pulsatile liver are signs of RV failure with systemic venous congestion l On percussion, hyper resonance of the lungs may be a sign of underlying COPD l ascites seen in severe disease

On auscultation of the chest wheezes & crackles: signs of underlying lung disease in

On auscultation of the chest wheezes & crackles: signs of underlying lung disease in early stages 1. Splitting of the S 2 2. Loud P 2

in advanced disease 1. sharp ejection click (single or multiple) over the pulmonary artery

in advanced disease 1. sharp ejection click (single or multiple) over the pulmonary artery 2. Followed by ejection systolic murmur 3. Latter on: diastolic pulmonary regurgitation murmur (Graham steel) 4. may be S 3 &/or S 4 5. systolic murmur of tricuspid regurgitation

DIFFERENTIAL DIAGNOSIS l. Congestive (biventricular) heart failure l. Primary pulmonic stenosis l. Primary pulmonary

DIFFERENTIAL DIAGNOSIS l. Congestive (biventricular) heart failure l. Primary pulmonic stenosis l. Primary pulmonary hypertension l. Right-sided heart failure due to congenital heart diseases l. Right heart failure due to right ventricular infarction

INVESTIGATIONS

INVESTIGATIONS

Routine investigation: l Hematocrit l> 50 polycythemia l > 60 – indication for phlebotomy

Routine investigation: l Hematocrit l> 50 polycythemia l > 60 – indication for phlebotomy

To confirm diagnosis ECG l X ray chest l Echocardiography l Right heart catheterization

To confirm diagnosis ECG l X ray chest l Echocardiography l Right heart catheterization l

E C G in Cor pulomale

E C G in Cor pulomale

Electrocardiography (ECG) RVH or RV strain a. right axis deviation b. R/S amplitude ratio

Electrocardiography (ECG) RVH or RV strain a. right axis deviation b. R/S amplitude ratio in V 1 greater than 1 R/S amplitude ratio in V 6 less than 1 c. P-pulmonale -increase in P wave amplitude in leads 2, 3, and a. VF

d. incomplete or complete right bundle branch block (RBB), especially if pulmonary embolism is

d. incomplete or complete right bundle branch block (RBB), especially if pulmonary embolism is the underlying etiology e. low-voltage QRS because of underlying COPD with hyperinflation and increased AP diameter of the chest.

Chest roentgenography enlargement of the central pulmonary arteries with oligemic peripheral lung fields- per.

Chest roentgenography enlargement of the central pulmonary arteries with oligemic peripheral lung fields- per. pruning l right descending pulmonary artery > 16 mm l left pulmonary artery >18 mm in diameter l. RVH l

l. Elevated brain natriuretic peptide (BNP) level l. Earliest evidence of CCF a natural

l. Elevated brain natriuretic peptide (BNP) level l. Earliest evidence of CCF a natural mechanism to compensate for elevated pulmonary hypertension and right heart failure by a. promoting diuresis and natriuresis, b. vasodilating systemic and pulmonary vessels

Arterial blood gas tests provide important information about the level of oxygenation and type

Arterial blood gas tests provide important information about the level of oxygenation and type of acid-base disorder

To know the etiology l. P F T to confirm underlying lung disease l.

To know the etiology l. P F T to confirm underlying lung disease l. To exclude pulmonary thromboembolism l. Ventilation/perfusion (V/Q) scan or CT chest l. Hypercoagulability states evaluated by levels of l proteins C and S l antithrombin III l factor V Leiden l antinuclear antibody (ANA) level for collagen vascular disease such as scleroderma l serum alpha 1 -antitrypsin

l. Oxygen therapy l. Diuretics l. Vasodilators l. Digitalis l. Anticoagulation therapy are all

l. Oxygen therapy l. Diuretics l. Vasodilators l. Digitalis l. Anticoagulation therapy are all different modalities used in the long-term management of Chronic cor pulmonale

llong-term oxygen therapy can be considered even if l. Pa. O 2 is greater

llong-term oxygen therapy can be considered even if l. Pa. O 2 is greater than 55 mm Hg or l. O 2 saturation is greater than 88%. l( because of vasodilator effect on pulmonary arteries)

DIURETICS l. Right ventricular filling volume markedly elevated l. Diuretics may result in improvement

DIURETICS l. Right ventricular filling volume markedly elevated l. Diuretics may result in improvement of function of bo the right and left ventricles adverse effects. a. Excessive volume depletion can lead to a decline in cardiac output b. hypokalemic metabolic alkalosis lead to cardiac arrhythmia Diuretics needs to be used with caution

Vasodilator drugs In long-term management of chronic cor pulmonale have modest results 1. Calcium

Vasodilator drugs In long-term management of chronic cor pulmonale have modest results 1. Calcium channel blockers l oral sustained-release nifedipine l diltiazem 2. beta blockers 3. Nitrates 4. angiotensin-converting enzyme (ACE) inhibitors l not routinely used. A trial of vasodilator therapy considered in patients with COPD with disproportionately high pulmonary blood pressure – more than 40 mm Hg

NEWER VASODILATORS lendothelin receptor antagonist l(Bosentan) lprostacyclin PGI 2 analogues l. Epoprostenol -i. v.

NEWER VASODILATORS lendothelin receptor antagonist l(Bosentan) lprostacyclin PGI 2 analogues l. Epoprostenol -i. v. liloprost - M D I THEY HAVE SHOWN A PROMISING EFFECT IN REDUCING THE PULMONARY HYPERTENSION

CARDIAC GLYCOSIDES NOT ROUTINELY INDICATED l. Beneficial effect not as obvious as in LVF

CARDIAC GLYCOSIDES NOT ROUTINELY INDICATED l. Beneficial effect not as obvious as in LVF l modest effect of digitalis on failing right ventricle in chronic cor pulmonale l. Must be used cautiously lshould not be used during the acute phases of respiratory insufficiency l. Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias

Theophylline l bronchodilatory effect l reduce pulmonary vascular resistance and pulmonary arterial pressures l

Theophylline l bronchodilatory effect l reduce pulmonary vascular resistance and pulmonary arterial pressures l weak inotropic effect and thus may improve right and left ventricular ejection l Strenghtens diaphragm l Stimulates the respiratory centre

Phlebotomy l Mean Pul art press and PVR decrease in polycythemic patients after phlebotomy

Phlebotomy l Mean Pul art press and PVR decrease in polycythemic patients after phlebotomy (hematocrit of >60 or 65) l The reduction of markedly elevated hematocrit level to about 50% by phlebotomy leads to 1. Reduction of blood viscosity 2. Reduction in PVR and pulmonary art pr 3. Improve gas exchange & increases exercise tolerance