Pulmonary hypertension: Clinical sciences

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Introduction0:00–0:43

Pulmonary hypertension or ph for short refers to an abnormally high pressure in the pulmonary circulation, which is divided into five main groups based on etiology group.
One refers to pulmonary arterial hypertension group. Two is pulmonary hypertension due to left heart disease group.
Three, pulmonary hypertension is associated with chronic lung disease or hypoxia group. Four, pulmonary hypertension is due to pulmonary artery obstruction.
And finally, group five is pulmonary hypertension with unclear or multifactorial mechanisms. Now, if your patient presents with a chief concern, suggesting pulmonary hypertension perform an ABCDE assessment to determine if they are unstable or stable, if unstable, stabilize their airway breathing and circulation, which might require endotracheal intubation and mechanical ventilation.

Unstable0:43–1:26

Next, obtain IV access and put them on continuous vital sign monitoring as well as cardiac telemetry and if needed. Don't forget to provide supplemental oxygen.
Finally, in some cases, you might need to place an indwelling pulmonary artery catheter, also known as a Swan Ganz catheter.
Next, obtain a focused history and physical exam. Your patient will typically report shortness of breath and chest discomfort while their physical exam will usually reveal hypotension and tachycardia.

Unstable - Focused H&P1:26–2:03

You might also notice signs of right sided heart failure, such as a right ventricular heave jugular venous distension with hepatojugular reflux, lower extremity, edema and cool extremities.
On auscultation. You might notice a loud pee two or a hollow systolic murmur from tricuspid regurgitation at this point, suspect cardiogenic shock.

Cardiogenic shock2:03–3:04

Then order labs including BNP as well as a 12 lead ECG chest X ray and echocardiogram labs will show an elevated BNP ECG will likely reveal right axis deviation peaked P waves on lead V two indicating right arterial enlargement and R waves greater than seven millimeters in lead V one consistent with right ventricular hypertrophy.
The chest X ray often reveals cardiomegaly. Finally, echocardiogram usually shows elevated estimated pulmonary artery pressure and right ventricular strain with hypertrophy or dilation at this point.
Diagnosed cardiogenic shock due to pulmonary hypertension. Once you diagnose cardiogenic shock due to pulmonary hypertension, proceed with management.

Management3:04–3:57

This primarily relies on decreasing right ventricular preload with loop diuretics such as furosemide along with sodium and fluid restriction.
If diuretics are ineffective, you may also consider ultra filtration. Next, decrease pa afterload using short acting inhaled vasodilators such as nitric oxide.
Next, try to increase heart contractility using inotropes like dobutamine and milrinone and vasopressors like norepinephrine.
In severe cases, you might need advanced interventions like mechanical circulatory support and extracorporeal membrane oxygenation.

Stable - H&P3:57–4:45

Ok. Now, let's go back to the ABCDE assessment and discuss stable patients.
First obtain a focused history and physical exam. Your patient is likely to report dyspnea at rest and with exertion, fatigue, chest discomfort, and sometimes even syncope.
The physical exam will show signs of right sided heart failure such as jugular venous distension, possibly with hepatojugular reflux and lower extremity edema.
Additional physical exam findings might include right ventricular heave on auscultation. You may hear a loud P two right sided S3 and hollow systolic murmur with these findings suspect pulmonary hypertension and obtain a 12 lead ECG chest X ray and echocardiogram.

12- lead ECG, Chest X-Ray, Echocardiogram4:45–5:33

On ECG you're likely to see right axis deviation, right atrial enlargement and right ventricular hypertrophy. While the chest X ray commonly shows cardiomegaly.
Finally, the echocardiogram typically reveals an elevated estimated pulmonary artery systolic pressure, right ventricular strain tricuspid regurgitation as well as right atrial and right ventricular enlargement.
With these findings diagnose pulmonary hypertension. Next, assess the underlying cause and categorize your patient's pulmonary hypertension by etiologic group using the World Health Organization classification to do this order a chest CT scan, CT pulmonary angiogram, pulmonary function tests sleep study and a ventilation perfusion scan.

WHO classification5:33–6:02

In some cases, you may also need a right or left heart catheterization. First, let's start with group one, which refers to pulmonary arterial hypertension or PH.

Group I6:02–8:09

For short, these patients have thick and narrow arteries which can be idiopathic or due to various conditions. For example, a mutation of the BMPR two gene which provides instructions for growth and differentiation of cells, including those in the walls of the small arteries of the lungs connective tissue conditions like scleroderma HIV, liver disease and even certain medications and toxins including stimulants like amphetamines and tyrosine kinase inhibitors like dasatinib to diagnose group one pulmonary hypertension.
Your patient should have no evidence of left heart disease, chronic lung disease, hypoxia, and chronic thromboembolic disease.
Ok. To confirm the diagnosis, the right heart catheterization must demonstrate elevated mean pulmonary artery pressure, typically 20 millimeters of mercury or more a pulmonary vascular resistance of two wood units or more.
And a normal pulmonary capillary wedge pressure typically less than 15 millimeters of mercury. With these findings diagnose pulmonary arterial hypertension.
Ok. Moving on to management, initiate treatment with supplemental oxygen loop diuretics like furosemide and a pulmonary rehabilitation program.
Additionally, administer vasoactive agents beginning with calcium channel blockers like Nifedipine. If calcium channel blockers are not effective.
Consider PDE five inhibitors like sildenafil endothelin receptor antagonists like Bostan prostacyclin like trip prosy insoluble guanylate cyclase stimulators like riociguat, moving on to group two, which refers to pulmonary hypertension due to left heart disease that's associated with increased postcapillary, pulmonary venous pressures.

Group II8:09–9:36

History is notable for hypertension, coronary artery disease, congestive heart failure or valvular heart disease. The ECG might reveal Q waves suggesting prior infarcts as well as left ventricular hypertrophy and left atrial enlargement.
The chest X ray is likely to show pulmonary edema and cardiomegaly. Additionally, the echocardiogram usually reveals left sided heart failure, either with reduced or preserved ejection fraction and left atrial enlargement.
There might also be left sided valvular dysfunction. In some individuals you might need to perform a left heart catheterization which would reveal coronary artery disease.
With these findings diagnose pulmonary hypertension due to left heart disease. Next, initiate management consisting of supplemental oxygen loop diuretics, cardiopulmonary rehabilitation and treatment of the underlying cause of left heart disease.
Ok. Let's move on to group three, which is pulmonary hypertension due to chronic lung disease or hypoxia which results in hypoxic pulmonary vasoconstriction.

Group III9:36–10:38

These patients usually have a history of pulmonary disease such as COPD or interstitial lung disease or they may have obstructive sleep apnea.
Pulmonary function testing generally shows obstructive or restrictive lung disease. While ct of the chest also reveals features of pulmonary parenchymal pathology.
A sleep study may reveal obstructive sleep apnea if the diagnosis is not already established with these findings diagnose pulmonary hypertension due to chronic lung disease or hypoxia.
In this case management involves supplemental oxygen loop diuretics, pulmonary rehabilitation and treatment of the underlying cause.

Group IV10:38–12:06

Ok. Now, let's turn our attention to group four, which is pulmonary hypertension due to pulmonary artery obstruction.
This is almost always due to pulmonary embolism that increases the pulmonary vascular resistance. So this group is often called chronic thromboembolic pulmonary hypertension or cteph.
For short, many patients report a history of venous thromboembolism and their CT pulmonary angiogram usually reveals organized thrombi throughout the pulmonary arteries, meaning that old unresolved clots become fibronic, resulting in obstruction, inflammation and vascular remodeling.
However, the CT pulmonary angiogram alone is not always sensitive enough to establish the diagnosis. So the preferred test is ventilation perfusion or VQ scan, which will show a VQ mismatch with these findings diagnose pulmonary hypertension due to pulmonary artery obstruction management involves supplemental oxygen and anticoagulation.
Usually with a direct oral anticoagulant like rivaroxaban. Don't forget a loop diuretic.
If there's volume overload. In many cases, this condition can be cured with a pulmonary artery thromboendarterectomy.
So, obtain a surgical consultation. Finally, let's discuss group five, which is pulmonary hypertension with unclear or multifactorial mechanisms.

Group V12:06–13:42

In this case, your patient may report a history of conditions such as sarcoidosis, glycogen storage disease, sickle cell disease, thyroid disorders, and dialysis dependent end stage renal disease.
And just like with group one pah, your patient should have no evidence of left heart disease, chronic lung disease, hypoxia, and chronic thromboembolic disease.
Now, to distinguish group five from group one, perform a right heart catheterization which typically reveals an elevated mean pulmonary artery pressure of 20 millimeters of mercury or more.
Additionally, pulmonary vascular resistance and pulmonary capillary wedge pressure will be normal treatment of group five.
Pulmonary hypertension is supplemental oxygen loop diuretics, pulmonary rehabilitation and treatment of any contributing underlying causes.
Now, here's one final clinical pearl. Once you initiate the management, many patients with pulmonary hypertension remain symptomatic if this is the case, obtain a surgical consultation for possible lung transplantation.
If left heart disease is contributing to the pulmonary hypertension, heart and lung transplantation may even be considered.

Review13:42–14:21

All right. As a quick recap, pulmonary hypertension refers to an abnormally high pressure in the pulmonary circulation, which is divided into five main groups based on etiology group.
One refers to pulmonary arterial hypertension group. Two is pulmonary hypertension due to left heart disease group.
Three, pulmonary hypertension is associated with chronic lung disease or hypoxia group. Four, pulmonary hypertension is due to pulmonary artery obstruction.
And finally, group five is pulmonary hypertension with unclear or multifactorial mechanisms.