DRG Category: 315
Mean LOS: 3.6 days
Description: Medical: Other Circulatory System Diagnoses With Complication or Comorbidity
Cor pulmonale is a disorder of the structure and function of the right side of the heart caused by a disease of the respiratory system, primarily pulmonary hypertension. Cor pulmonale is estimated to cause approximately 5% to 7% of all types of heart disease in adults, and chronic obstructive pulmonary disease (COPD) due to chronic bronchitis or emphysema is the causative factor in more than 50% of people with cor pulmonale. While it is difficult to know how many people in the United States have cor pulmonale, experts estimate that 15 million people have the condition. The right ventricle is a thin-walled chamber as compared to the left ventricle and is more responsive to volume changes. With increased resistance in the pulmonary system, the right side of the heart works harder, the systolic pressure rises, the right ventricle dilates, and ultimately, right-sided heart failure occurs.
A number of physiological changes lead to poor gas exchange. Alveolar wall damage results in anatomic reduction of the pulmonary vascular bed as the number of pulmonary capillaries are reduced and the vasculature stiffens from pulmonary fibrosis. Constriction of the pulmonary vessels and hypertrophy of vessel tissue are caused by alveolar hypoxia and hypercapnia. Abnormalities of the ventilatory mechanics bring about compression of pulmonary capillaries. Cor pulmonale accounts for approximately 25% of all types of heart failure. Complications of cor pulmonale include biventricular heart failure, hepatomegaly, pleural effusion, and thromboembolism related to polycythemia.
In addition to COPD, acute cor pulmonale is produced by a number of other pulmonary and pulmonary vascular disorders but primarily by acute respiratory distress syndrome (ARDS) and pulmonary embolism. Two factors in ARDS lead to right ventricular overload: the disease itself and the high transpulmonary pressures that are needed to treat ARDS with mechanical ventilation. In the United States, approximately 25,000 sudden deaths occur per year from heart failure associated with pulmonary emboli. Other conditions can also lead to cor pulmonale. Respiratory insufficiency—such as chest wall disorders, upper airway obstruction, obesity hypoventilation syndrome, and chronic mountain sickness caused by living at high altitudes—can also lead to the chronic forms of the disease. It can also develop from lung tissue loss after extensive lung surgery. A contributing factor is chronic hypoxia, which stimulates erythropoiesis, thus increasing blood viscosity. Cigarette smoking is also a risk factor.
No clear genetic contributions to susceptibility have been defined.
Middle-aged and older men are more likely to experience cor pulmonale, but incidence in women is increasing. In children, cor pulmonale is likely to be a complication of cystic fibrosis, hemosiderosis, upper airway obstruction, scleroderma, extensive bronchiectasis, neurological diseases that affect the respiratory muscles, or abnormalities of the respiratory control center.
The Centers for Disease Control and Prevention report that 11.5% of White persons, 9.5% of Black persons, 7.4% of Hispanic persons, and 6.0% of Asian persons have heart disease. Significant health disparities exist in the cardiac care of underrepresented groups as compared to White persons. Black, Indigenous, and other people of color are known to receive care less often guided by standard cardiac care guidelines than White persons. Unless patients have health insurance, White patients are more likely to receive coronary angiograms and other coronary interventions than Black and Hispanic patients. Black, Indigenous, and other people of color are also less likely to be referred to cardiologists and cardiac surgeons than White persons (Batchelor et al., 2019). COPD is a leading cause of hospitalization for Veterans. During military deployment, soldiers are often exposed to dust, chemicals, smoke, and fumes for long periods of time. Cigarette smoking rates are high among active and retired military.
Transgender is a term used to describe persons whose gender identity is different from their sex assigned at birth. Approximately 1% of the U.S. population identify themselves as transgender. Sexual and gender minority persons have higher odds for multiple chronic conditions, cancer, and poor quality of life, and are more apt to have disabilities than cisgender males and females (cisgender is a term used to describe persons whose gender identity and gender expression are aligned with their assigned sex listed on their birth certificate). Gender-affirming hormone therapy is the use of hormone therapy for gender transition or gender affirmation and can be masculinizing or feminizing. It may also affect cardiovascular health in transgender females. In a large sample, researchers have found that transgender men and women are more likely to be overweight than cisgender women. Compared to cisgender women, transgender women reported higher rates of diabetes, ischemic stroke, angina/coronary disease, and myocardial infarction. Gender-nonconforming men and women reported higher odds of myocardial infarction than cisgender women. Transgender women also had higher rates of any cardiovascular disease than cisgender men (Cacerese, Jackman, et al., 2020; Connelly et al., 2019). While large-scale studies are not available, these factors may place some sexual and gender minority persons at risk for cor pulmonale.
The prevalence of cor pulmonale around the world depends on the prevalence of cigarette smoking and other tobacco use, air pollution, toxic exposure, and other risk factors for lung diseases. Global data are not available from developing countries.
PLANNING AND IMPLEMENTATION
Collaborative
The primary goals of treatment for cor pulmonale are to manage the underlying lung disease, improve oxygenation, and increase right ventricular contractility. The patient with an acute exacerbation of cor pulmonale requires mechanical ventilation and is usually admitted to an intensive care unit. Patients admitted with heart failure related to ARDS or pulmonary embolism who require specialized treatment, such as hemodynamic monitoring, may also be admitted to a special care unit.
Specific medical treatment for cor pulmonale consists of reversing hypoxia with low-flow oxygen and improving right ventricular function, depending on the underlying cause. In the case of acute cor pulmonale associated with pulmonary emboli, higher concentrations of oxygen may be used. The physician seeks to correct fluid, electrolyte, and acid-base disturbances and may prescribe fluid and sodium restrictions to reduce plasma volume and the work of the heart. In the setting of right ventricular failure, therapies may include vasopressor medications and fluid loading to maintain blood pressure. Single-lung or double-lung transplantation may be considered for people with terminal disease.
Supportive Care
Respiratory therapists provide bronchodilator therapy and may need to teach or reinforce the patient's use of breathing strategies. Therapists may also teach energy conservation. A dietitian confers with the patient and family about the need for low-sodium foods and small, nutritious servings. Specific nutritional deficiencies may need to be corrected as well. Depending on the derivation of cor pulmonale, fluids need to be limited to 1,000 to 1,500 mL per day to prevent fluid retention. Social service agencies will probably be needed for a consultation as well because cor pulmonale creates long-term disability with the likelihood that the patient has not been employed for some time. Unless the patient is old enough to receive Medicare, hospitalization costs are a serious concern.
| Medication or Drug Class | Dosage | Description | Rationale |
|---|
| Calcium channel blockers | Varies with drug | Nifedipine, diltiazem | Lower pulmonary pressures |
| Bronchodilators | Varies with drug | Beta2-adrenergic agonists, anticholinergics | Relieve bronchospasm |
Other Drugs: Diuretics are used when right ventricular pressures are elevated, but they are used cautiously so that cardiac output does not decrease. Massive pulmonary embolism may require thrombolytic agents. Oxygen therapy, vasodilators, low-dose digitalis, theophylline, antidysrhythmic agents, prostacyclin analogues and receptor agonists, endothelin receptor antagonists, and anticoagulation therapy may be used in long-term management.
Independent
The patient requires bedrest and assistance with the activities of daily living if hypoxemia and hypercapnia are severe. Provide meticulous skin care. Reposition the bedridden patient frequently to prevent atelectasis. Reinforce proper breathing strategies for the patient: breathe in through the nose and out slowly through pursed lips, using abdominal muscles to squeeze out the air; inhale before beginning an activity and then exhale while doing the activity, such as walking or eating.
Nurses can teach patients to control their anxiety, which affects their breathlessness and fear. Teach the patient the use of relaxation techniques. Because patients are continually breathless, they become anxious if they feel rushed; focus on providing a calm approach. Help reduce the patient's fear of exertional dyspnea by providing thoughtful care that builds trust. Encourage the patient to progress in small increments.
Because of the exertion that talking requires, many patients with cor pulmonale may not be able to respond adequately in conversation. Try to understand the patient's reluctance to “tire out” and become familiar with reflective techniques that allow a patient to respond briefly. Integrate your teaching into the care to avoid the need to give the patient too much information to assimilate at the time of discharge.
Evidence-Based Practice and Health Policy
Mandoli, G., Sciaccaluga, C., Bandera, F., Cameli, P., Esposito, R., D'Andrea, A., Evola, V., Sorrentino, R., Malagoli, A., Sisti, N., Nistor, D., Santoro, C., Bargagli, E., Mondillo, S., Galderisi, M., & Cameli, M. (2021). Cor pulmonale: The role of traditional and advanced echocardiography in the acute and chronic settings. Heart Failure Reviews, 26, 263–275.
- The authors explained the value of echocardiography in diagnosing cor pulmonale and creating a therapeutic plan. They provided an overview of the current standards of an echocardiographic evaluation in both acute and chronic cor pulmonale, focusing also on the findings in the most common pathologies causing this condition.
- Although the distinction between acute and chronic cor pulmonale is primarily based on taking the patient's history and performing a clinical examination, echocardiography can provide additional information about right ventricular morphology and function. In the context of both acute and chronic cor pulmonale, echocardiography also can provide information to help in determining particular treatments as well as evaluating their success.