Kembara Xtra - Medicine - Cor Pulmonale

Kembara Xtra - Medicine - Cor Pulmonale 
​Introduction 
The words "cor" and "pulmonale" come from the Latin for "heart" and "lungs," respectively, giving rise to the medical term "cor pulmonale." As a result, cor pulmonale is a type of pulmonary hypertension that occurs as a cardiac consequence of primary pulmonary illness.
Acute or chronic pulmonary processes have the potential to lead to elevated pressures on the right side of the heart. As a direct consequence, the development of pulmonary hypertension (PH) can lead to the formation of anatomical abnormalities and/or the impairment of the function of the right ventricle (RV).
pulmonary hypertension (PH) is diagnosed when the resting mean pulmonary artery pressure (mPAP) is lower than 25 mm Hg as evaluated by right cardiac catheterization.
pulmonary hypertension (PH) is a condition that can be caused by abnormalities in the pulmonary system, such as conditions that affect the lung parenchyma, pulmonary circulation, chest wall, and/or ventilatory systems. Both physiologically and clinically, the pathophysiologic mechanisms behind pulmonary arterial hypertension (WHO Group I) and pulmonary hypertension (PH) secondary to pulmonary processes are distinct from one another. As a result, pulmonary arterial hypertension, which belongs to WHO Group I, will not be taken into consideration as a potential cause of cor pulmonale for the purposes of this review.
It is possible for cor pulmonale to develop in either an acute or chronic context.
- Acute: a sudden rise in pulmonary arterial pressure that can lead to RV overload, malfunction, and even possible collapse of the cardiovascular system
- Chronic: gradual hypertrophy and dilatation of the RV over the course of several months to years, ultimately leading to dysfunction and the possibility of failure

The study of epidemiology, including incidence and prevalence. 
6–7 percent of all types of adult heart disease that can occur in the United States.
The incidence of chronic obstructive pulmonary disease (COPD) varies greatly across the globe as a result of air pollution, tobacco usage, and toxic exposure.
The most prevalent cause of cor pulmonale is chronic obstructive pulmonary disease (COPD), which is responsible for an estimated 10–30% of heart failure admissions in the United States.

Incidence 
Estimation is difficult, but the best we can do is 1 in 10,000 to 3 in 10,000 per year.
Prevalence
Assessment is difficult; the best estimate is between 2/1,000 and 6/1,000.
Acute: A sudden occurrence, such as a massive pulmonary embolism (PE), increases resistance to blood flow in the pulmonary vasculature. This causes a quick and severe increase in pressure proximal to the right ventricular outflow tract. Chronic: This condition occurs over a long period of time and can be caused by a number of factors. It's possible that the RV won't be able to create enough force to counteract this pressure, which will result in a reduced cardiac output from the RV. This, in turn, would lead to a lower cardiac output from the left ventricle (LV). When combined with a low cardiac output, elevated RV pressures have the potential to produce coronary ischemia, which in turn can further reduce cardiac output and eventually lead to full collapse of the cardiovascular system.
persistent: a condition of the pulmonary system that causes persistent hypoxia and eventually leads to vasoconstriction of the pulmonary vasculature. An increase in pulmonary vascular resistance is the result of the progressive hypertrophy of the pulmonary artery system and the dysregulation of intrinsic vasoactive processes, which are mediated by nitric oxide, cyclooxygenase, and endothelin. In addition, the evidence suggests that both the capillary and postcapillary pulmonary vasculature are involved, albeit to various degrees, in all of the PH groups.

pulmonary hypertension is the result of increased pulmonary vascular resistance. PH delivers greater pressures and volumes to the thin-walled, low-pressure RV, inducing maladaptive remodeling (concentric hypertrophy, followed by eccentric dilation). This maladaptive remodeling is typically coupled with tricuspid regurgitation, which leads to eventual deterioration in RV systolic and diastolic performance.
In these patients, indicators that may point to the presence of PH include an abnormally low diffusing capacity of the lungs for carbon monoxide (DLCO) and a high partial pressure of carbon dioxide (pCO2).
Pulmonary diseases - Lung parenchymal disease, including chronic obstructive pulmonary disease (COPD), also known as interstitial lung disease (ILD), and pulmonary fibrosis.
- Thromboembolic illness of the pulmonary circulation (related with WHO Group IV pulmonary hypertension)
- Conditions affecting the chest wall: extreme obesity and kyphoscoliosis
– Ventilation: obstructive sleep apnea (OSA) and obesity hypoventilatory syndrome; neuromuscular illnesses such as Guillain-Barré syndrome, muscular dystrophy, myasthenia gravis, and spinal cord injuries. – Obesity hypoventilatory syndrome (OHS).
Cor pulmonale is not thought to be brought on by failure of the left ventricle of the heart.

Risk Factors 
pulmonary embolism (PE) is the most prevalent underlying cause of acute cor pulmonale. underlying pulmonary disorders are the most common cause of chronic cor pulmonale.
- Factors that increase the likelihood of developing pulmonary diseases
○ Tobacco usage (COPD)
○ Occupational exposures (ILD)
a condition known as hypercoagulability, also known as chronic thromboembolic illness
Chest wall and ventilation problems brought on by old age and obesity.

Prevention 

Management of underlying pulmonary diseases, particularly vigorous correction of hypoxia and acidosis, which may contribute to progressive PH if left untreated.

Conditions That Often Occur Together 

PH is characterized by the presence of a resting mPAP that is greater than 25 mm Hg.

DIAGNOSIS HISTORY
Dyspnea is the most typical manifestation of this condition. Dyspnea is a symptom that can be present at rest, during physical activity, or as paroxysmal nocturnal dyspnea, despite the fact that it is nonspecific.
Other pulmonary symptoms include pleuritic chest pain, cough, and hemoptysis; general heart failure symptoms include fatigue, lethargy, and syncope; exertional angina is less likely; right-sided heart failure symptoms include anorexia, early satiety, digital cyanosis, clubbing, right upper quadrant discomfort (hepatic congestion), and lower extremity edema; general heart failure symptoms include fatigue, lethargy, and syncope;
Hoarseness caused by compression of the left recurrent laryngeal nerve by enlarged pulmonary arteries. Cardiovascular collapse, shock, and/or cardiac arrest may occur in acute or advanced chronic settings. Hoarseness caused by compression of the left recurrent laryngeal nerve by enlarged pulmonary vessels.

The Patient's Clinical Examination 
Although it does not definitively diagnose right-sided heart failure, the most prevalent symptom of this condition is peripheral edema.
Symptoms that are general include a pale complexion, profuse sweating, clubbing, cyanosis, and tachypnea.
jugular venous distention, with a strong a-wave in the neck. tachypnea, wheezing, and shallow breathing in the lungs.
Heart – Increased intensity of the pulmonic component of the second heart sound (P2) – Splitting of S2 over the cardiac apex with inspiration – Audible right-sided S3 or S4 – RV heave – Pansystolic murmur heard best at right midsternal border increasing with inspiration, consistent with tricuspid regurgitation (typically a late sign) – Pansystolic murmur heard best at right midstern
● Abdomen: hepatomegaly

Clubbing, cyanosis, bilateral lower extremity edema, and may also display indications of deep vein thrombosis (DVT), such as soreness or unilateral swelling Extremities: clubbing, cyanosis, and bilateral lower extremities edema


Differential Diagnosis 
Other reasons of failure on the right side of the heart include: Failure on the left side of the heart
Right-sided intrinsic cardiomyopathy WHO Groups I, II, and V for PH

Results From the Laboratory 

The initial diagnostic test of choice is a two-dimensional echocardiography.
- Pressures in the pulmonary arteries that are too high
– Enlargement of the right ventricle; – Protrusion of the interventricular septum into the left ventricle during systole; – Flattening of the interventricular or interatrial septum; – Right ventricular dilation and hypokinesis; – Hypertrophy of the right ventricle
– Tricuspid regurgitation – Dilation of the right atrium – Acute thromboembolic pulmonary illness as demonstrated by right ventricular hypokinesis with sparing of the apex (McConnell sign) – Main pulmonary artery to ascending aorta diameter ratio more than one – Main pulmonary artery to ascending aorta diameter ratio less than one
Depending on the picture quality or the operator, echocardiography has the potential to either overestimate or underestimate the pulmonary arterial pressures. Right heart catheterization is the best method for confirming the arterial pressures in the pulmonary circulation.
MRI is the most accurate method for diagnosing emphysema and ILD. It is able to evaluate heart pressures, size, function, myocardial mass, and viability. If echocardiography is unable to provide a definitive diagnosis, MRI can serve as a replacement.
Catheterization of the right ventricle is the gold standard for diagnosing pulmonary hypertension and is therefore essential for diagnosing cor pulmonale.
– An increased central venous pressure (CVP) – A mean PA pressure of less than 25 mm Hg while the patient is at rest Initial Tests (laboratory, imaging)
Because of the prolonged hypoxia, a CBC may demonstrate symptoms of polycythemia.
It's possible that a low cardiac output will cause an elevated creatinine level to show up on a basic metabolic panel (BMP).
Abnormal liver function tests (LFTs) could be the result of either proximal hepatic congestion or inadequate distal cardiac output, both of which are caused by RV failure.
Because of the strain on the right ventricle, levels of brain natriuretic peptide (BNP) and cardiac troponin can become increased.

The presence of underlying thromboembolic pulmonary illness may be indicated by a positive D-dimer test result. Arterial blood gas results may indicate hypercapnia because of COPD or hypoxemia because of ILD.
Patients with COPD have arterial blood gases that demonstrate a decreased PaO2 along with normal or elevated PaCO2. The electrocardiogram frequently reveals symptoms of right-sided hypertrophy.
- Deviation from the right axis
- An R/S wave ratio in V1 that is greater than 1
- Hypertrophy of the right ventricle (R wave in leads V1 and V2 with S waves in leads V5 and V6)
– Enlargement of the right atrium, as shown by the presence of a P pulmonale (increased amplitude of the P wave in leads II, III, and avF, with a "tent-like" appearance)
– Right bundle branch block that is either incomplete or complete. – S1S2S3 pattern or S1Q3T3 inverted pattern.
x-ray of the chest reveals cardiomegaly, which is an enlargement of the central pulmonary arteries and a reduction in the size of the vessels in the periphery (oligemia).
On lateral images, there is a decreased amount of retrosternal space due to an enlarged right ventricle.
- The right heart border becomes more prominent as a result of the enlargement of the right atrium.
Spiral computed tomography (CT) scan of the chest – Diagnosis of acute PE – Diagnosis of COPD and ILD – Evidence of PE (Westermark sign, Fleischner sign, and Hampton hump) – Evidence of structural illness (i.e., kyphosis) – Evidence of PE (Westermark sign, Fleischner sign, and Hampton hump) – Evidence of structural disease (i.e., kypho
Ventilation/perfusion (V/Q) scan: – High specificity and sensitivity for acute and chronic thromboembolic disease – Screening method of choice for chronic thromboembolic PH because of its higher sensitivity compared with CT pulmonary angiogram – May be used for diagnosis of acute thromboembolic disease if contraindication to chest spiral: – High specificity and sensitivity for acute and chronic thromboembolic disease – High Confirmation of a diagnosis of chronic thromboembolic illness (WHO Group IV PH) with pulmonary angiography may be required after receiving a CT scan.
pulmonary angiography is the diagnostic test that is considered to be the gold standard for chronic thromboembolic pulmonary illness.
Polysomnography is the diagnostic gold standard for obstructive sleep apnea

pulmonary function tests (often abbreviated as PFTs)
- DLCO: A decrease in lung volume in conjunction with a lower capacity for carbon monoxide diffusion could be an indication of ILD. COPD is linked to lower levels of diffusing capacity for carbon monoxide.
- Problems breathing that are either obstructive or restrictive (such as ILD, chest wall abnormalities, and COPD)
- A lower overall exercise capacity as measured by cardiopulmonary exercise testing
- Significantly worse than predicted impairment in gas exchange both when the patient is at rest and while they are exercising
TREATMENT
Reduce symptoms, boost quality of life, and boost survival rates all at the same time. Oxygenation, preservation of heart function, and pH reduction all contribute to a reduction in the burden of disease.

Preventive Measures in General 

Treat the underlying disease — Long-acting bronchodilators, long-acting antimuscarinic medicines, and/or inhaled corticosteroids may be effective for the long-term disease management of underlying pulmonary disease.
– Anticoagulation therapy may be necessary for patients who have an underlying chronic thromboembolic condition.
Complementary and alternative medicine as warranted
Hypoxia and other sleep problems might benefit from treatment with continuous positive airway pressure or bilevel positive airway pressure.
– Patients who suffer from neuromuscular disease should get ventilation either through the use of positive-pressure masks, negative-pressure body suits, or through mechanical ventilation.
– In cases of severe polycythemia or signs and symptoms of hyperviscosity (hematocrit greater than 55%), phlebotomy may be an appropriate treatment option.

Medication
Oxygen Patients with COPD and cor pulmonale who are hypoxemic have a better chance of surviving when they get long-term, continuous oxygen therapy.
– Patients diagnosed with PH who have a PaO2 that is continuously lower than 55 mm Hg or a saturation that is lower than 88% at rest, while sleeping, or while ambulating should be given oxygen in order to maintain an O2 level that is higher than 92 mm Hg.
– Avoiding situations in which you are exposed to high altitude is important.
In order to keep oxygen saturations at a level greater than 91% while at an elevated altitude or while flying, supplemental oxygen should be given as necessary.
Dobutamine and milrinone are two inotropes that might help enhance cardiac output. This would be beneficial for the preservation of heart function.
Diuretics lower right ventricular filling pressures and lessen peripheral edema caused by right heart failure.
It is recommended that excessive volume depletion be avoided.
It is important to keep a watchful eye out for metabolic alkalosis since it has the potential to inhibit ventilatory drive and add to hypoxia.
Reduce pH levels – The treatment of the underlying condition is the defining characteristic of management (2) [C].
– When a condition is unresponsive to conventional medical treatment, advanced therapies may be helpful, despite the lack of evidence supporting their use.
– Riociguat is an option for patients who have pulmonary hypertension that is linked with chronic thromboembolic conditions (WHO Group IV).


Considerations Prior to Referral 
Patients who have been diagnosed with cor pulmonale ought to be directed to a specialized clinic for the purpose of receiving expert assistance.

Surgical Methods and Operations 

Endarterectomy is considered the gold standard treatment for chronic thromboembolic illness, which falls under WHO Group IV. Nevertheless, around one third of individuals in this group are determined to have an inoperable condition. – In individuals who are unable to undergo surgery, balloon pulmonary angioplasty is a treatment option that has a minimal risk of death and may lead to an improvement in hemodynamic measures.

Lung volume reduction surgery, often known as LVRS, is a surgical option that may be helpful for patients with advanced upperlobe predominant emphysema who have poor disease control while receiving maximal medicinal therapy. LVRS is a surgical option that may be beneficial for individuals.
Lung and/or heart transplantation may be necessary for patients with moderate to severe diseases that are unresponsive to treatment with medicines.

Ongoing Medical Attention 

Restrictions on salt intake and fluid intake

It is strongly suggested that smoking be stopped, and that secondary smoke be avoided as much as possible.
Having a conversation with your doctor about your activity level is highly recommended.
It is best to avoid becoming pregnant.

Prognosis 

Patients who suffer cor pulmonale as a result of COPD have a higher risk of passing away compared to patients who only have COPD. There is a 50% chance of survival after 5 years for those who have COPD and mild illness (PAP 20 to 35 mm Hg).