Cor Pulmonale


Article Author:
Daniel Garrison


Article Editor:
Jawedulhadi Memon


Editors In Chief:
Jesse Cole


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
2/15/2019 4:12:38 PM

Introduction

Cor pulmonale is a Latin word that means "pulmonary heart," its definition varies, and presently, there is no consensual definition.

Cor pulmonale can be defined as an alteration in the structure (e.g., hypertrophy or dilatation) and function of the right ventricle (RV) of the heart caused by a primary disorder of the respiratory system resulting in pulmonary hypertension.

Right-sided heart failure secondary to left-sided heart failure, or congenital heart disease is not considered cor pulmonale.[1][2][3][4]

Etiology

Pulmonary hypertension is associated with diseases of the lung (e.g., chronic obstructive pulmonary disease, interstitial lung disease), vasculature (e.g., idiopathic pulmonary arterial hypertension), upper airway (e.g., obstructive sleep apnea), or chest wall (e.g., kyphoscoliosis). Diseases that damage lungs are autoimmune (e.g., scleroderma), cystic fibrosis, and obesity hypoventilation syndrome are also lead to pulmonary hypertension.

Massive pulmonary embolism is the most common cause of acute cor pulmonale.

A massive pulmonary embolism can mimic a myocardial infarction with elevated troponins, ST changes, chest pain, and shortness of breath.

Chronic obstructive pulmonary disease (COPD) is the most common cause of cor pulmonale. The severity of cor pulmonale appears to correlate with the magnitude of hypoxemia, hypercapnia, and airflow obstruction.

In most patients with COPD, cor pulmonale tends to be accompanied by mild pulmonary hypertension (i.e., mean pulmonary artery pressure 40 mmHg or less).

Epidemiology

The exact prevalence of cor pulmonale is difficult to determine, as physical examination and routine tests are relatively insensitive for the detection of pulmonary hypertension and RV dysfunction. Cor pulmonale is estimated to account for 6% to 7% percent of all types of adult heart disease in the United States. Globally, the incidence of cor pulmonale varies widely among countries, depending on the prevalence of cigarette smoking, air pollution, and other risk factors for various lung diseases.

Pathophysiology

The pathophysiology of cor pulmonale is a result of increased right-sided filling pressures from pulmonary hypertension that is associated with diseases of the lung.[5][6][7]

Under normal physiologic conditions, the right ventricle pumps against a low-resistance circuit.

Normal pulmonary vascular resistance is approximately one-tenth the resistance of the systemic arteries. Chronic hypoxemia leading to chronic vasoconstriction produces smooth muscle proliferation in small pulmonary arteries. Hypoxemia produces changes in vascular mediators such as Nitric Oxide, Endothelin1 (ET1) and platelet-derived growth factors (PDGf A and B). Nitric oxide is a vasodilator; hypoxemia reduces endothelial cell production of nitric oxide and results in impaired smooth ms relaxation.

The initial pathophysiologic event in the production of cor pulmonale is an elevation of the pulmonary vascular resistance. As the resistance increases, the pulmonary arterial pressure rises, and the right ventricular work increases leading to right ventricular enlargement (e.g., thickening, dilation, or both).

History and Physical

Symptoms may include dyspnea on exertion (most common), fatigue, lethargy, exertional syncope and exertional chest pain, abdominal edema or distension, and lower extremity edema.

The clinical signs occur late, being observed at an advanced stage of the disease far after the development of pulmonary hypertension.

Physical findings may include: 

  • Jugular venous distension: Prominent jugular V wave, indicating the presence of tricuspid regurgitation
  • Peripheral (ankle) edema: The best sign of RHF, but it is not specific and can arise from other causes
  • Cardiovascular: Palpable left the parasternal lift, loud S2 (accentuation of the pulmonary component of the second heart sound) narrow splitting of S2, a holosystolic murmur of tricuspid regurgitation at the left lower sternal border, right-sided S4 heart sound
  • Abdomen: Hepatomegaly, ascites.

Evaluation

Laboratory investigations are directed toward defining the potential underlying etiologies as well as evaluating the complications of cor pulmonale.[8][9][10] These include:

  • Chest radiograph: Enlargement of pulmonary artery may be seen, cardiomegaly is confined predominantly, if not exclusively, to the right ventricle and other features may be detected according to the cause
  • Electrocardiogram: Shows features of right ventricular hypertrophy/enlargement
  • Doppler echocardiography (most practical but heavily operator dependent): The non-invasive diagnosis of pulmonary hypertension is presently based on echocardiography. Continuous-wave Doppler echocardiography allows the calculation of the transtricuspid pressure gradient from the peak velocity of the tricuspid regurgitant jet
  • Chest CT angiography to rule out pulmonary thromboembolism as a cause. Main pulmonary artery diameter measurements greater than 29 mm have a sensitivity of 84% and specificity of 75% for the diagnosis of pulmonary hypertension
  • Ventilation/perfusion (V/Q) scanning can be particularly useful in evaluating patients with cor pulmonale, especially if pulmonary hypertension is due to chronic thromboembolic pulmonary hypertension (CTEPH)
  • MRI: This noninvasive technique yields highly accurate dimensions of the right ventricle but is not routinely used
  • PFTs and 6-minute walk test for assessment of severity of lung disease and exercise capacity respectively
  • A right heart cath is a gold standard for diagnosis, assessment of Pulmonary hypertension severity. Right heart catheterization reveals evidence of right ventricular (RV) dysfunction (mean pulmonary artery pressure (PAP) above 25 mmHg) without left ventricular (LV) dysfunction. Differentiating left-sided from the right-sided disease includes measuring the pulmonary capillary wedge pressure (PCWP), which is an estimation of left atrial pressure. Thus, RV dysfunction is also defined as having a PCWP below 15 mmHg.

Treatment / Management

Treatment is aimed primarily at treating the underlying condition; the aim is improving oxygenation and right ventricular (RV) function by increasing RV contractility and decreasing pulmonary vasoconstriction.

Oxygen therapy relieves hypoxemic pulmonary vasoconstriction, which then improves cardiac output, lessens sympathetic vasoconstriction, alleviates tissue hypoxemia, and improves renal perfusion.

Diuretics are used to decrease the elevated right ventricular (RV) filling volume in patients with chronic cor pulmonale.

The use of cardiac glycosides, such as digitalis in patients with cor pulmonale has been controversial, and the beneficial effect of these drugs is not as obvious as in the setting of left heart failure. Nevertheless, studies have confirmed a modest effect of digitalis on the failing right ventricle in patients with chronic cor pulmonale.

Pearls and Other Issues

Prognosis: 

The prognosis of cor pulmonale is variable-dependant upon the underlying pathology. Development of cor pulmonale as a result of a primary pulmonary disease usually heralds a poorer prognosis.

Highlights:

  • Cor pulmonale is right ventricular dysfunction from long-standing pulmonary HTN.
  • All primary lung diseases can cause PHTN and thus cor pulmonale.
  • Dyspnea on exertion is the most common symptom.
  • Primarily, treatment aims to treat the underlying condition.

Enhancing Healthcare Team Outcomes

The diagnosis and management of cor pulmonale is by a multidisciplinary team that consists of a cardiologist, internist, radiologist, pulmonologist and an intensivist. The treatment is aimed primarily at treating the underlying condition; the aim is improving oxygenation and right ventricular (RV) function by increasing RV contractility and decreasing pulmonary vasoconstriction. The outlook for patients with cor pulmonale is dependent on the primary condition, which if not controlled, leads to a poor outcome. Most of the patients who do recover have a long protracted course marked by relapse of symptoms. The quality of life for most patients is poor.[11] (Level V)

 

 


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Cor Pulmonale - Questions

Take a quiz of the questions on this article.

Take Quiz
What is the definition of cor pulmonale?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What is the most common cause of acute cor pulmonale in North America?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What is the best test to confirm cor pulmonale?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Cor pulmonale may be suspected on chest radiography if the pulmonary artery diameter is greater than what length?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
All of the following are true regarding cor pulmonale except:



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following regarding cor pulmonale is false?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following is not a cause of cor pulmonale?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What physical finding not seen in cor pulmonale?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A male with a 40-pack per year history complains of dyspnea on exertion. Chest x-ray shows signs of chronic obstructive pulmonary disease and enlargement of the right ventricle. Which of the following is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following is associated with cor pulmonale?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which clinical sign is associated with cor pulmonale?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 60-year-old male heavy smoker presents due to progressive dyspnea and bilateral lower limb swelling. bilaterally. Arterial blood gases show pH 7.24, PCO2 79, HCO3 29. What is the acid-base abnormality?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Cor Pulmonale - References

References

Vieira JL,Távora FRF,Sobral MGV,Vasconcelos GG,Almeida GPL,Fernandes JR,da Escóssia Marinho LL,de Mendonça Trompieri DF,De Souza Neto JD,Mejia JAC, Chagas Cardiomyopathy in Latin America Review. Current cardiology reports. 2019 Feb 12;     [PubMed]
George PM,Patterson CM,Reed AK,Thillai M, Lung transplantation for idiopathic pulmonary fibrosis. The Lancet. Respiratory medicine. 2019 Feb 6;     [PubMed]
Niwa K, Aortic dilatation in complex congenital heart disease. Cardiovascular diagnosis and therapy. 2018 Dec;     [PubMed]
Neidenbach R,Niwa K,Oto O,Oechslin E,Aboulhosn J,Celermajer D,Schelling J,Pieper L,Sanftenberg L,Oberhoffer R,de Haan F,Weyand M,Achenbach S,Schlensak C,Lossnitzer D,Nagdyman N,von Kodolitsch Y,Kallfelz HC,Pittrow D,Bauer UMM,Ewert P,Meinertz T,Kaemmerer H, Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy. Cardiovascular diagnosis and therapy. 2018 Dec;     [PubMed]
Lee S, Comprehensive Nursing Management for Valvular Disease. Critical care nursing clinics of North America. 2019 Mar;     [PubMed]
Yoon YS,Jin M,Sin DD, Accelerated lung aging and chronic obstructive pulmonary disease. Expert review of respiratory medicine. 2019 Feb 8;     [PubMed]
Smolders VF,Zodda E,Quax PHA,Carini M,Barberà JA,Thomson TM,Tura-Ceide O,Cascante M, Metabolic Alterations in Cardiopulmonary Vascular Dysfunction. Frontiers in molecular biosciences. 2018;     [PubMed]
Patel S,Cole AD,Nolan CM,Barker RE,Jones SE,Kon S,Cairn J,Loebinger M,Wilson R,Man WD, Pulmonary rehabilitation in bronchiectasis: a propensity-matched study. The European respiratory journal. 2019 Jan;     [PubMed]
Balsam P,Ozierański K,Kapłon-Cieślicka A,Borodzicz S,Tymińska A,Peller M,Marchel M,Crespo-Leiro MG,Maggioni AP,Drożdż J,Opolski G,Grabowski M, Differences in clinical characteristics and one-year outcomes of hospitalized heart failure patients in succeeding European Society of Cardiology-Heart Failure Registries - Pilot and Long-Term. Polish archives of internal medicine. 2019 Jan 16;     [PubMed]
van Cleemput J,Sonaglioni A,Wuyts WA,Bengus M,Stauffer JL,Harari S, Idiopathic Pulmonary Fibrosis for Cardiologists: Differential Diagnosis, Cardiovascular Comorbidities, and Patient Management. Advances in therapy. 2019 Feb;     [PubMed]
Kim M,Tillis W,Patel P,Davis RM,Asche CV, Association between asthma/chronic obstructive pulmonary disease overlap syndrome and healthcare utilization among the US adult population. Current medical research and opinion. 2019 Jan 5;     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Radiology Tech-Cardiac US. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Radiology Tech-Cardiac US, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Radiology Tech-Cardiac US, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Radiology Tech-Cardiac US. When it is time for the Radiology Tech-Cardiac US board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Radiology Tech-Cardiac US.