Pulmonary HTN and Cor pulmonale Flashcards
What is the definition of pulmonary hypertension (PH)?
Mean pulmonary arterial pressure (mPAP) at or greater than 20 mmHg at rest.
What are the clinical signs associated with pulmonary hypertension?
Progressive exertional dyspnea, presyncope, syncope, or exertional angina.
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Signs of right heart failure such as peripheral edema, elevated JVP, IVC Engorgement , loud P2, right sided S3, right ventricular heave, holosystolic tricuspid regurg murmur.
What are the major concerns of right sided heart congestion associated with pulmonary hypertension?
Venous congestion and IVC Engorgement: Patients experience symptoms of high right atrial pressure, such as peripheral edema, abdominal distension, early satiety, ascites, congestive hepatopathy, and oliguria (cardiorenal).
When patients with pulmonary hypertension start to experience presyncope or syncope, what does this say about their heart condition?
In pulmonary hypertension, presyncope with the Valsalva maneuver (coughing/defecation) likely reflects poor RV output (rather than a ventricular arrhythmia). This is due to limited cardiac output. Cardiac output cannot be sufficiently augmented during exercise, leading to exertional fatigue and syncope/presyncope. Marginal RV function renders patients susceptible to vagal bradycardia and hypotension (eg, defecation and cough syncope, stooping to tie shoes).
What would be seen on CXR in a patient with pulmonary HTN?
Bilateral hilar enlargement. The high pulmonary artery pressure leads to dyspnea and chest tightness and are manifestations of vessel wall stretch. Rarely, hemoptysis may occur.
What does an ECG do to help render a diagnosis of pulmonary hypertension?
ECG will show a right axis deviation. Additionally, patients with pulmonary hypertension can experience increased RV demand ischemia, whereas the LV is solely perfused in diastole, the RV is perfused during systole. Therefore the right side of the heart is more susceptible to low stroke volume. Patients may experience exertional chest pain related to episodes of RV ischemia, further contributing to RV decline.
Is biopsy ever indicated with pulmonary hypertension?
Diagnosis of pulmonary hypertension is based on clinical presentation, noninvasive tests, and hemodynamic data (eg, echocardiography). Tissue sampling typically adds no further information and carries a high risk for complications from biopsy (eg, high-pressure bleeding), so essentially, NO!
How is pulmonary hypertension diagnosed?
A transthoracic echocardiogram is used as a non-invasive evaluation of pressures initially. There will be evidence of right ventricular enlargement as well as right atrial enlargement. An ECHO is followed by a right heart catheterization, as a right heart cath is the most accurate diagnostic test.
What is the gold standard for diagnosing pulmonary hypertension?
Right heart catheterization.
What are the five WHO classifications of pulmonary hypertension?
- Pulmonary arterial hypertension (PAH)
- Left heart disease (mitral stenosis)
- Lung disease/chronic hypoxemia
- Chronic thromboembolic pulmonary hypertension (CTEPH)
- Multifactorial or unclear mechanisms.
What is the most common cause of pulmonary hypertension?
WHO class III; Lung diseases such as COPD, interstitial lung disease, and obstructive sleep apnea.
If the cause for pulmonary hypertension is idiopathic, what class of pulmonary hypertension does this belong to?
This is class I pulmonary hypertension.
Drugs/toxins (e.g., amphetamines), toxins, HIV, schistosomiasis, belong to which class of pulmonary hypertension?
Class I
If pulmonary arterial hypertension (PAH) appears to have a familial component, which gene is implicated and what class of pulmonary hypertension does this belong to?
BMPR-2 mutation, Class I pulmonary hypertension.
Connective tissue diseases (e.g., scleroderma, CREST) are associated to which class of pulmonary hypertension?
Class I
What are the initial elements for working up Group 1 PAH?
- check family history
- screen and treat underlying autoimmune disease (connective tissue disease)
- evaluate for drugs or toxins (check urine for amphetamines)
- test for infections (schistosomiasis) if indicated
- management of HIV
How is Group 2 PH managed?
Perform an ECHO and optimize left ventricular function (e.g., heart failure therapy) and treat significant valve disease such as mitral stenosis. A normal left ventricular function on echocardiography and normal pulmonary capillary wedge pressure (<18 mm Hg) rule out PH due to left-sided heart failure. If this is the underlying cause for pulmonary hypertension, treatment would involve addressing either valvular disease, heart failure, or reduced ejection fraction with valve replacement, or heart failure medications such as a renin-angiotensin inhibitors or cardioselective beta blocker.
What causes Group 3 pulmonary hypertension?
Chronic lung diseases (e.g., COPD, interstitial lung disease) or chronic hypoxemia (e.g., obstructive sleep apnea).
What would be the workup for Group 3 PAH?
Pulmonary function testing, chest imaging, or polysomnography, would serve to either rule in or out PH due to lung disease or sleep-disordered breathing (eg, sleep apnea).
How is Group 3 PH managed?
Treat the underlying respiratory condition (e.g., bronchodilators for COPD, CPAP for OSA). PH due to lung disease and/or chronic hypoxemia (group 3) is treated by addressing the underlying lung conduction (eg, inhaled muscarinic antagonist for chronic obstructive pulmonary disease)
What causes Group 4 pulmonary hypertension (CTEPH)?
Chronic thromboembolic disease, typically after multiple pulmonary embolisms (PEs).
How is Group 4 CTEPH managed?
Evaluation would require a V/Q scan or a pulmonary angiography. This is treated with Pulmonary thromboendarterectomy surgery (curative) and lifelong anticoagulation.
What are the general measures for managing all forms of pulmonary hypertension?
Maintain normoxia, euvolemia, and sinus rhythm; contraceptive counseling; immunizations; cardiac rehabilitation.
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For all forms of pulmonary hypertension, general supportive measures aim to maintain physiologic homeostasis (ie, normoxia, euvolemia, sinus rhythm). Furthermore, the following components of care include, avoiding pregnancy due to maternal and fetal mortality remaining unacceptably high (25%-50%), even with optimal pharmacotherapy. Right ventricular (RV) failure and obstructive physiology are especially dangerous for normal peripartum volume shifts and anesthesia. Most pulmonary vasoactive medications are pregnancy class X teratogens (absolutely contraindicated). Guidelines issue a strong recommendation to avoid pregnancy, advising non-estrogen-based contraception (lower risk for venous thrombosis for women of childbearing age. Regular aerobic exercise in a supervised cardiac/pulmonary rehabilitation program, aerobic exercise, as tolerated, is safe and effective for restoring functional status. Strenuous weightlifting and other Valsalva maneuvers inhibiting venous return should be avoided. Provide nutritional support with a dietary plan to regulate salt and fluid intake and achieve weight loss or gain (eg, pulmonary cachexia), targeting a healthy BMI. Patients should be referred to an accredited PH program due to the superior outcomes. Such centers can offer psychosocial counseling and assistance to cope with the many dimensions of PH, including family planning.
What hematologic disorder can lead to pulmonary hypertension?
Polycythemia vera. This is Class 5 PAH (multifactorial).
What is the definitive treatment for refractory pulmonary hypertension?
Bilateral lung transplantation.
What dangerous cardiac condition is secondary to chronic pulmonary hypertension?
Right sided heart failure, cor pulmonale.
What is cor pulmonale?
Isolated right heart failure caused by advanced pulmonary hypertension due to mechanisms other than left heart failure.
What are the clinical features of cor pulmonale?
Exertional dyspnea, angina, syncope, signs of right heart failure (JVD, peripheral edema), RV heave, hepatomegaly, ascites, abdominal distention, and early satiety.
