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Pulmonary Hypertension

PNS SHIFA

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Syed Intisar Hussain

Overview

Known to damage the right side of the heart, pulmonary hypertension (PH) refers to the high blood pressure in the blood vessels supplying blood to the lungs i.e., pulmonary arteries. The thickening of these arteries often causes this.&nbsp;&nbsp;

&nbsp;

Pulmonary hypertension on its own causes a lot of issues for the heart, causing your heart to overwork itself to ensure the adequate flow of blood. The condition is known to get worse over time with no known cure.

Pulmonary hypertension is an uncommon disease found in only 15 out of 50 million people in the US. Women are more commonly known to get affected<a href="#accordion">[1]</a>. The same ratio also applies to the people of Pakistan.

In technical terms, blood pressure greater than 25 mmHg at rest, and 30 mm Hg during exercise qualifies to be checked for pulmonary hypertension. The biggest concern is that this can be life-threatening and cause right ventricular failure<a href="#accordion">[2]</a>. In many cases, pulmonary hypertension is a complication or a symptom of another disease, regardless, it requires immediate medical attention. Especially in people with severe mitral valve disease, where the development of pulmonary hypertension is very likely (100%).

<div>&nbsp;</div>

Instead of types, pulmonary hypertension is divided into five groups. These groups differentiate the disease based on its origin and the root cause. These include:

&nbsp;

<ul>
	<li>
	<h5>Group 1: Pulmonary Arterial Hypertension (PAH)</h5>
	</li>
</ul>

This refers to the narrowing of the pulmonary arteries by any mechanism which restricts the blood flow. This restricted blood flow causes the right side of the heart to work harder to overcompensate for less blood being supplied to the lungs. Idiopathic PAH occurs when there is no known cause of pulmonary hypertension. Whereas heritable PAH refers to when the PAH is hereditary, and its occurrence is a result of your genes<a href="#accordion">[3]</a>.

&nbsp;

<ul>
	<li>
	<h5>Group 2: Pulmonary Hypertension Due To Left Heart Disease</h5>
	</li>
</ul>

According to WHO, group 2 of pulmonary hypertension results from issues on the left side of the heart. Unlike pulmonary arterial hypertension, the walls of the arteries are not the problem or the causative agent here. The issue is the beating of the heart on the left side, and the left side&rsquo;s inability to keep up with pumping the blood. Thus, causing an increase in the pressure<a href="#accordion">[4]</a>.

&nbsp;

<ul>
	<li>
	<h5>Group 3: Pulmonary Hypertension Due To Lung Diseases And/Or Hypoxemia</h5>
	</li>
</ul>

People that fall under this group of pulmonary hypertension have contracted the issue due to some other lung disease, for example, COPD or emphysema. These diseases cause the airways to narrow, making it difficult to exhale. Restrictive lung diseases are characterized by the individual having issues inhaling, similar to interstitial lung disease or pulmonary fibrosis. Sleep apnea, where the individual suffers from obstructive breathing patterns, can also be a cause of pulmonary hypertension<a href="#accordion">[3]</a>. These issues decrease the oxygen supply to the lungs, causing arteries to tighten and increasing blood pressure in the lungs.

&nbsp;

<ul>
	<li>
	<h5>Group 4: Pulmonary Hypertension Due To Chronic Blood Clots In The Lungs</h5>
	</li>
</ul>

This group is also referred to as chronic thromboembolic pulmonary hypertension (CTEPH). As the name suggests, the body is unable to dissolve blood clots in the lungs, leading to the formation of scar tissue and blocking normal blood flow. This puts a burden on your heart&rsquo;s right side, causing it to work harder. This type of PAH is believed to be cured by the removal of the blood clot from the arteries, resuming blood flow. The process is called pulmonary thromboendarterectomy. The catch is that the eligibility criteria for this surgery are very specific, and most patients do not check all the boxes. However, the surgery is not a promised solution for PH, as it may linger in your body even after the removal of the clot<a href="#accordion">[4]</a>.

&nbsp;

<ul>
	<li>
	<h5>Group 5: Pulmonary Hypertension Due To Unknown Causes</h5>
	</li>
</ul>

Conditions such as sickle cell anemia, chronic hemolytic anemia, splenectomy, and metabolic disorders are causative factors, but no known connection or mechanism explains the occurrence of pulmonary hypertension in these cases<a href="#accordion">[3]</a>.

<div>&nbsp;</div>

Signs And Symptoms

The initial symptoms of pulmonary hypertension are mild and can even go unnoticed by the individual since it involves mere shortness of breath. The symptoms develop and worsen over time, and tend to get more severe if left untreated. These symptoms include<a href="#accordion">[5]</a>:

<ul>
	<li><a href="https://www.merisehat.pk/disease/angina" target="_blank">Chest pain</a></li>
	<li>Shortness of breath</li>
	<li>Bluish tinge of skin and lips</li>
	<li>Loss of appetite</li>
	<li>Dizziness&nbsp;</li>
	<li>Fatigue</li>
	<li>Increased heartbeat</li>
	<li>Pain in the abdomen</li>
	<li>Edema</li>
</ul>

&nbsp;

In cases of pulmonary arterial hypertension, the symptoms are not as profound and the disease is mainly caught in the severe stages<a href="#accordion">[6]</a>.

<div>&nbsp;</div>

Causes

With so many diseases leading to the development of pulmonary hypertension, some fundamental factors come into play regarding its cause. These include:

<ul>
	<li>Diseases that restrict oxygen supply to the lungs&nbsp;</li>
	<li>Issues causing narrowing of the pulmonary arteries or shortening of the branches</li>
	<li>Diseases or conditions that affect the left side of the heart&rsquo;s ability to pump blood</li>
	<li>Possible blood clots that can block or narrow down the blood vessels</li>
</ul>

<div>&nbsp;</div>

Earlier diagnosis is believed to significantly increase the survival rate in people suffering from pulmonary hypertension. Initially, concluding just based on the symptoms that the individual has pulmonary hypertension is very less likely. Especially, due to the unique nature of the disease. Patients with systemic sclerosis are commonly screened for pulmonary hypertension due to the high occurrence. It was found that around 100% of individuals manage to live a year more after their early diagnosis if the right steps are taken to care for the issues<a href="#accordion">[7]</a>.

&nbsp;

Echocardiography Doppler Transthoracic Echocardiography (TTE) is a recommended screening tool, identified and approved by the joint European Society of Cardiology/ European Respiratory Society. The TTE echocardiography measures systolic pulmonary artery pressure (PAP), a huge indicator of how well your heart is performing. Alongside that, a higher tricuspid regurgitation velocity is also associated with the possible presence of pulmonary hypertension<a href="#accordion">[8]</a>.

<div>&nbsp;</div>

Diagnosis

Due to its rare or uncommon nature, pulmonary hypertension is not always on top of the list of differential diagnoses, especially, if it is idiopathic pulmonary hypertension. However, in cases of other diseases like systemic sclerosis, it is the first consideration. Your healthcare worker will start the diagnosis by first asking about your symptoms, family history, current medication, and any ongoing or previous related medical condition(s).&nbsp;

An echocardiogram that utilizes sound waves to estimate the pressure in your arteries tells the practitioner how well your heart is performing. Another diagnostic tool is the usage of a catheter on the right side of the heart. This involves the insertion of the catheter into your arteries, all the way up to your heart to measure the blood pressure there.

A six-minute walk test may also be performed to monitor your breathing capability, after a little exercise, followed by an electrocardiogram (ECG or EKG)<a href="#accordion">[9]</a>.

Once pulmonary hypertension is confirmed, the next step is to determine the causative factor which is done by performing computerized tomography (CT Scan) and pulmonary function tests<a href="#accordion">[7]</a>.

&nbsp;

<h5>Classes Of Pulmonary Hypertension</h5>

There are a total of 4 classes based on the severity of your pulmonary hypertension<a href="#accordion">[10]</a>:

<ul>
	<li>The very first class includes the occurrence of symptoms only when the individual is performing physical activities.</li>
	<li>The second class refers to when basic physical movements cause issues like chest pain and tiredness, but these symptoms are not present at rest.&nbsp;</li>
	<li>The smallest of movements, like moving your arms, causing distress in breathing and other symptoms is a sign of the third class of pulmonary hypertension.</li>
	<li>The fourth class refers to the triggering of symptoms while resting, and the worsening of symptoms with physical movement.</li>
</ul>

<div>&nbsp;</div>

Treatment

Since there is no known cure for pulmonary hypertension, the treatment is symptomatic. With the right medications and practices, you can slow down its progress, and increase longevity and the quality of your life.&nbsp;

The treatments required by pulmonary hypertension are long-term and need to be followed up every once in a while. However, in cases where pulmonary hypertension is caused due to some other factor, the treatment is more focused on curing the underlying causes<a href="#accordion">[9]</a>.&nbsp;

&nbsp;

<h5>Medications&nbsp;</h5>

The medicines used for the treatment of pulmonary hypertension include<a href="#accordion">[11]</a>:

&nbsp;

<ul>
	<li>Anticoagulants &ndash; This is to thin down your blood for a better flow, and avoid the formation of any clots.</li>
</ul>

&nbsp;

<ul>
	<li>Diuretics &ndash; Also known as water pills, they aid the kidney in removing excess fluid from the body, thus, reducing the heart&rsquo;s burden.</li>
</ul>

&nbsp;

<ul>
	<li>Vasodilators &ndash; These are a staple in any hypertension-related issues. As the name suggests, these medicines aid in the dilation of vessels, allowing better passage of blood from the heart.&nbsp;</li>
</ul>

&nbsp;

<ul>
	<li>Guanylate Cyclase Stimulators &ndash; Known for increasing the concentration of nitric oxide in the body, these lead to a relaxation of the pulmonary arteries and help lower the pressure in the lungs.</li>
</ul>

&nbsp;

<ul>
	<li>Calcium Channel Blockers &ndash; They stop the contraction of the muscles surrounding the walls of the blood vessels.</li>
</ul>

&nbsp;

<h5>Surgical Solutions</h5>

In severe cases, especially in younger patients with idiopathic pulmonary arterial hypertension, there are only two options available - a lung or a heart transplant<a href="#accordion">[11]</a>. If the disease does not respond to medicines, an atrial septostomy, which is an open-heart surgery characterized by the creation of an opening between the left and right atria of the heart to reduce pressure on the right side of the heart, may be conducted<a href="#accordion">[12]</a>.&nbsp;

<div>&nbsp;</div>

There are many complications associated with pulmonary hypertension. These include:

&nbsp;

<ul>
	<li>Heart Enlargement: The right side of the heart may increase in size, especially the lower right chamber. The ventricle enlarges due to the increased load on the arteries.</li>
</ul>

&nbsp;

<ul>
	<li>Heart Failure: Due to the mechanical strain applied on the heart, as a result of increased burden, the right ventricle&rsquo;s walls thicken and expand. But it causes even more distress to the heart, leading to eventual failure of the right ventricle, or the entire heart<a href="#accordion">[13]</a>.</li>
</ul>

&nbsp;

<ul>
	<li>Blood Clots: There is an increased risk of developing blood clots due to pulmonary hypertension.</li>
</ul>

&nbsp;

<ul>
	<li>Arrhythmias: Cardiac arrhythmias are a common complication of pulmonary hypertension, but some can be life-threatening.&nbsp;</li>
</ul>

&nbsp;

<ul>
	<li>Lung Bleeding: This is a life-threatening complication, and may even cause the individual to cough up blood.</li>
</ul>

<div>&nbsp;</div>

The following are the risk factors that contribute to the development of pulmonary hypertension in individuals:

<ul>
	<li>Eisenmenger syndrome, which&nbsp;is a congenital heart defect characterized by a hole in the heart, causing the mixing of the blood and leading to increased pressure in the pulmonary arteries</li>
	<li>Family history of pulmonary hypertension</li>
	<li>Obesity</li>
	<li>Blood clotting issues</li>
	<li>Congenital heart defects</li>
	<li>Usage of illegal substances or even weight loss medications</li>
	<li>Taking serotonin reuptake inhibitors (SRIs)</li>
</ul>

<div>&nbsp;</div>

There is no exact way to prevent the occurrence of pulmonary hypertension. However, incorporating healthier lifestyle choices, managing high blood pressure, ensuring a healthy weight and managing any chronic lung disease can aid in either delaying or possibly preventing the issue.

&nbsp;

Pulmonary hypertension has always been associated with possible issues in the lungs or the heart, but other factors at play have rarely been discussed. A recent article highlighted that in PH there is reduced cerebral blood flow, even at rest and even more during physical movements. Furthermore, the impact of PH on renal circulation, metabolic, and endocrine dysfunction, as well as skeletal and respiratory musculature dysfunction has also been discussed<a href="#accordion">[14]</a>.&nbsp;

&nbsp;

Key Points

<ul>
	<li>Pulmonary hypertension is a rare disorder that involves a build-up of blood pressure in the pulmonary arteries.</li>
	<li>There are 5 groups of pulmonary hypertension based on the causative factors.&nbsp;</li>
	<li>The symptoms commonly include shortness of breath, inability to perform rigorous or physically demanding tasks, chest pain, a bluish tinge of the skin, and even loss of appetite.</li>
	<li>Pulmonary hypertension could be idiopathic with no known cause, or in many cases, it is caused due to some other underlying disorder.</li>
	<li>There is no cure but the symptoms are treated using a wide array of medicines. In terms of surgical options, lung or heart transplant and atrial septostomy may be performed, but none of these procedures or treatments promises a permanent solution.</li>
	<li>Pulmonary hypertension cannot be avoided, but by adopting a healthier lifestyle, even with other diseases, there is a good chance of delaying its onset.</li>
	<li>Heart enlargement, heart failure, arrhythmias and blood clots are some of the common complications of pulmonary hypertension.</li>
</ul>

<div>&nbsp;</div>

[1] Deborah Jo Levine. Pulmonary arterial hypertension: updates in epidemiology and evaluation of patients. The American Journal of Managed Care. 2021 Mar 1;27(Suppl 3):S35–41.

[2] Kristin E Schwab. Pulmonary Arterial Hypertension. Medscape. 2022.

[3] PH Association. About Pulmonary Hypertension. PH Association.

[4] Pulmonary Associates. 5 Pulmonary Hypertension Groups You Should Know About. Pulmonary Associates.

[5] Cleveland Clinic. Pulmonary Hypertension. Cleveland Clinic. 2022.

[6] NHS. Pulmonary Hypertension. NHS. 2020.

[7] Vachiery JL, Coghlan G. Screening for pulmonary arterial hypertension in systemic sclerosis. European Respiratory Review. 2009 Sep 1;18(113):162–9.

[8] Kiely DG, Lawrie A, Humbert M. Screening strategies for pulmonary arterial hypertension. European Heart Journal Supplements. 2019 Dec 1;21(Supplement_K):K9–20.

[9] UCSF Health. Pulmonary Hypertension Diagnosis. UCSF Health.

[10] NHS. Diagnosis - Pulmonary Hypertension. NHS. 2020.

[11] Mayo Clinic. Pulmonary Hypertension. Mayo Clinic. 2020.

[12] NHS. Treatment - Pulmonary hypertension. NHS. 2020.

[13] Mak SM, Strickland N, Gopalan D. Complications of pulmonary hypertension: a pictorial review. The British Journal of Radiology. 2017 Feb;90(1070):20160745.

[14] Nickel NP, Yuan K, Dorfmuller P, Provencher S, Lai YC, Bonnet S, et al. Beyond the Lungs: Systemic Manifestations of Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. 2020 Jan 15;201(2):148–57.