Pulm HTN/ Cor. Pulm Flashcards
Define pulmonary HTN
Heterogeneous group of disorders characterized by elevated mean pulmonary arterial pressure:
PH = mean pulmonary arterial pressure > 20 mmHg @ rest during RH catheterization
OR
Mean pulmonary arterial pressure > 35 mmHg via non-invasive estimate (Transthoracic ECHO)
Pulmonary HTN
1) Normal pulmonary artery pressure
2) Chronic elevated pulmonary artery pressure leads to what?
3) Most common cause of pulmonary HTN is a downstream consequence of what?
1) 8 mmHg
2) Vessel remodeling, vasoconstriction, and increase vascular resistance
3) Cardiopulmonary disease; HF, COPD
List the WHO Classifications for pulmonary hypertension by group and give examples
Group 1: Pulmonary Arterial Hypertension (PAH)
Idiopathic – primary pulmonary HTN
Group 2: PH 2nd to left heart disease
Systolic, diastolic heart failure, valvular heart disease (mitral stenosis)
Group 3: PH 2nd to lung disease and hypoxia
COPD, OSA, ILD
Group 4: PH 2nd to chronic PE
Group 5: PH 2nd to multifactorial
Metabolic, systemic, and hematologic d/o (SSD) and others
Describe the pathophys Primary pulmonary HTN (PAH)
Group 1: PAH - progressive narrowing of distal pulmonary arteries via various pathologic insults such as vasoconstriction, medial hypertrophy, intimal proliferation and fibrosis
Describe Secondary PH
Group 2: PH (HF) primarily a venous process from passive congestion with vasoconstriction and venous remodeling
Group 3: PH (lung disease) destruction of the alveolar capillary beds or chronic hypoxic vasoconstriction
Group 4: (PE) subsequent vasoconstriction and remodeling of the pulmonary arterial bed
Group 5: secondary affects of systemic/autoimmune disease
Selected annual screening for PAH in what 2 groups? Give examples
Asymptomatic patients at increased risk
Carrier for bone morphogenic protein receptor type II gene mutation
1st degree relative with PAH
Systemic scleroderma
Pre-liver transplant w/u
Symptomatic patients at increased risk
Congenital heart disease
HIV
Methamphetamine use
Portal HTN
SSD
What is scleroderma, also known as systemic sclerosis?
Group of rare diseases that involve the hardening and tightening of the skin
Scleroderma also may cause problems in the blood vessels, internal organs and digestive tract
Pulmonary hypertensionPresentation
Non-specific
Similar to HF or valvular disease
Symptoms are chronic, progressive dyspnea with minor exertion, fatigue, and pre-syncope or syncope
Give the OLDCARTS for pulmonary HTN
Onset, Duration, Timing – chronic, progressive symptoms, may develop parallel to other chronic disease progression such as HF or COPD
Location – pulmonary circulation, depends on secondary cause/group
Characterization – progressive dyspnea , fatigue, symptoms associated with right heart failure (ascites, HJR, JVD, hepatomegaly, LE edema, prolonged cap refill)
Aggravating/Alleviating – underlying disease not well controlled/optimal management of secondary cause
Radiation – associated with CV, Pulmonary, PE, and systemic disease
Associated Symptoms – depends on cause – chest pain, palpitations, weight gain due to fluid retention
PH Examination
Exam findings generally related to affects of the underlying disease
Signs of RHF
JVD/HJR/hepatomegaly
Peripheral LE edema
Abdominal distention/ascites
Diagnostic evaluation in patient with dyspnea & suspect Pulmonary HTN: What are your 4 office based options, and what would each show?
1) CXR: RV enlargement, lung disease, prominent pulmonary arteries and congestion
2) ECG: findings suggestive of right ventricular hypertrophy - Cor Pulmonale, and P-Pulmonale.
3) Labs: elevated BNP (HF), troponins, D-dimer for DVT/PE,
4) PFT – normal spirometry, low DLCO
Specialty referral for suspected pulmonary HTN in a pt with dyspnea
If any combination of work up suggests PH, referral to specialist is indicated
Trans Thoracic Echocardiogram (TEE): best first look at pulmonary arteries, RH (> 35 mm Hg)
Right heart catheterization: gold standard (> 20 mm Hg)
Pulmonary HTNtreatment: PAH
Referral to pulmonology
Supplemental O2, anticoagulation?
Calcium channel blocker in appropriate patients, endothelin receptor antagonist, PDE-5 inhibitors (Viagra), etc.
You should ID and treat underlying causes of PH; give 4 examples
1) HF – will cover in Cardio
2) COPD – optimize treatment, smoking cessation
3) PE – anti-coagulate, treat cause if reversible (smoking, OCP, hypercoag state)
4) Treat underlying systemic disease
Pulmonary hypertension: What vaccines should pts get?
Annual flu vaccination
Pneumonia vax – PCV 20 or 21
COVID vaccination
RSV vaccination in select patients (60+)
Tdap
Contraception - pregnancy is currently discouraged (estrogen hypercoagulable)
Cor Pulmonale: Describe
Persistent PH …
Strains the thin-walled right ventricle … RVH, then dilation …
Ultimately right ventricle failure
Most common cause of death in patients with pulmonary HTN
Cor Pulmonale:
1) What are the 3 non-specific Sx?
2) What are the 3 signs of right heart failure?
1) DOE, Fatigue, Exercise intolerance
2) Lung: wheezing and crackles
Cardiovascular: cyanosis, parasternal lift, JVD/HJR, peripheral edema, digital clubbing
GI: hepatomegaly, HJR
Cor pulmonale: What should primary care do?
EKG – changes c/w right ventricular and atrial enlargement
CXR – enlarged heart
Right atria and ventricle
Enlarged pulmonary arteries
Cor pulmonale do: What should a specialist do?
Echocardiogram
Estimates pulmonary arterial pressure
Evaluates heart
Right heart catheterization
Describe Cor Pulmonale management
Optimal management of underlying pulmonary condition:
1) COPD – smoking cessation, home oxygen, Rx, non-invasive ventilatory support
2) Vasodilators – CCB
3) Consider anticoagulation due to increased risk of DVT/PE
4) Cautious diuretic therapy – right heart failure is pre-load dependent
PE (pulmonary embolism): most result from what that sends emboli to heart and on to lung?
Lower extremity DVT
What are symptoms of PE (pulmonary embolism)?
Acute onset
1) Dyspnea
2) Pleuritic CP
3) Cough
4) Hemoptysis
5) Syncope
(not specific)
What are Sx of a lower extremity DVT?
Acute onset of unilateral leg (calf) swelling and pain
Describe the presenting S/Sx of a PE (embolism)
Tachypnea, tachycardia, may be hypotensive/unstable
Chest exam - pleural friction rub, crackles/rales
Signs of DVT: unilateral leg swelling/pitting edema, tenderness, visible collateral vein
Signs of RV failure: JVD, systolic murmur left sternal edge, hepatomegaly