The siRNA-mediated knockdown of AP-1 restores the function of the pulmonary artery and the right ventricle by reducing perivascular and interstitial fibrosis and key molecular players in cardiopulmonary disease
Pulmonary Artery Aneurysm In Longstanding Idiopathic Pulmonary Arterial Hypertension
Figure
Computed tomography scan of the chest with contrast medium in the soft tissue window in a frontal plane. Red circle: main stem of the left coronary artery and proximal portion of the left anterior descending artery. Black asterisk: ectatic pulmonary trunk
A 54-year-old female patient with a known 20-year history of idiopathic pulmonary arterial hypertension (IPAH) presented as an emergency with typical angina pectoris symptoms as well as increasing dyspnea on exertion 5 years after her last outpatient appointment. In the absence of signs of myocardial ischemia in laboratory tests and on electrocardiography, chest imaging with computed tomography revealed an ectatic pulmonary trunk (Figure) with a maximum diameter >10 cm, prompting the suspicion that this was compressing the main stem of the left coronary artery. Coronary angiography with intravascular ultrasound was performed, ruling out coronary heart disease and main stem compression. Since angina-pectoris symptoms can also be caused by IPAH, we ultimately deemed this to be the cause of our patient's complaints. Following treatment optimization, the patient subsequently presented symptom-free. With an incidence of 2–5/1 000 000, the 5-year survival rate is approximately 50% with vasodilator treatment, depending on concomitant diseases. There are a number of case reports of pulmonary artery aneurysm in pulmonahypertension, which are seen increasingly often given the increasing life expectancy. The most important complications include dissections or ruptures, as well as compression of the bronchial system and/or the main stem of the left coronary artery.
Christopher Alexander Hinze, Dr. Med. Da-Hee Park, Dr. Med. Benjamin-Alexander Bollmann, Klinik für Pneumologie, Medizinische Hochschule Hannover, hinze.Christopher@mh-hannover.De
Conflict of interest statement: The authors state that no conflict of interest exists.
Translated from the original German by Christine Rye.
Cite this as: Hinze CA, Park DH, Bollmann BA: Pulmonary artery aneurysm in longstanding idiopathic pulmonary arterial hypertension.Dtsch Arztebl Int 2023; 120: 890a. DOI: 10.3238/arztebl.M2022.0404
What Is An Aneurysm?
If you get an aneurysm, it means you have a bulge in the wall of an artery. It happens when the pressure of blood passing through has forced a weakened part of the artery to balloon outward or when the blood vessel wall is weakened for a different reason.
Aneurysms can happen in any blood vessel, but they usually form in the belly or chest portions of your aorta -- the main blood vessel that carries blood from your heart -- or in arteries that nourish your brain.
Aneurysms there are serious, while those in other areas, such as your leg, can be less hazardous.
The most serious threat of an aneurysm is that it will burst and cause a stroke or massive bleeding, which can be life-threatening. A large aneurysm can affect your circulation and lead to blood clots.
It's important to get it diagnosed and treated early. Aneurysms often have mild symptoms or none at all, so routine exams can help your doctor check for warning signs.
Aortic aneurysm. As the name suggests, this type happens in your aorta. It can be linked with hardening of the arteries, also known as atherosclerosis. It may be an inherited condition or a complication of high blood pressure or smoking.
Cerebral aneurysm. Also known as a berry aneurysm, you get this kind in the wall of a blood vessel in your brain. Smoking raises your risk of getting one.
Popliteal artery. One of the more common peripheral vascular aneurysms, it is a bulging or weakness in the wall of the artery that supplies blood to the knee joint, calf and thigh.
Ventricular aneurysm. This is a bulge in the wall of your heart. A previous heart attack is the most common cause. In rare cases, a severe chest injury can also lead to it.
Any condition that causes your artery walls to weaken can bring one on. The most common culprits are atherosclerosis and high blood pressure.
Deep wounds and infections can also lead to an aneurysm. Or you may be born with weakness in one of your artery walls.
To diagnose an aneurysm, your doctor will ask you questions, including whether another member of your family has had one. Then, they'll give you a complete exam, during which they'll:
If your doctor thinks you have an aneurysm in your aorta, the main artery in your body, you may get an ultrasound test. This is painless and can pinpoint and measure an aneurysm. If they think one is in your chest, you might get a CT scan.
If your doctor is concerned that you have one in your brain, you may get a CT scan or an invasive test called an angiogram. During this, dye is injected into an artery in an arm or leg and travels to your brain. A picture of your brain is then taken. The dye will make it easier for your doctor to see any problems.
An MRI can also check your aorta or blood vessels in your brain.
The only way to treat an aneurysm is to have it repaired with surgery or an endovascular procedure.
Sometimes, surgery isn't possible, or it may pose more danger than the aneurysm. Careful monitoring and medication may be best in that case.
Your doctor will figure out the size, type, and location of the aneurysm. What they find will help determine your best treatment.
For inoperable aneurysms, you may be prescribed drugs to lower your blood pressure or ease the force of your heart's beating. Your chance of it bursting will go down.
Even for an operable aneurysm, your doctor may first try medication and a wait-and-see approach, monitoring its growth.
You may need surgery if your doctor finds that the aneurysm has become big enough to be dangerous. A surgeon can treat it by inserting a clip that cuts off blood flow to the affected area.
In some cases, the aneurysm can be removed. That section of artery can be replaced with a synthetic graft.
The most important thing you can do to prevent aneurysms is to control your blood pressure.
If you have a family history of stroke or heart disease, make changes in your diet and lifestyle to improve your health.
What To Know About A Fusiform Aneurysm
A fusiform aneurysm causes the artery to bulge or balloon on all sides. This type is less common than a saccular aneurysm, which causes a bulge on one side of the artery.
An aneurysm is a weakness in an artery wall that causes it to bulge.
Doctors may find it more difficult to treat a fusiform aneurysm, as this type does not feature a "neck" that they could seal off from the main artery. Because of this, fusiform aneurysms may present a higher risk of complications than saccular aneurysms.
An aneurysm occurs due to weakness in an arterial wall.
Common causes of fusiform aneurysms include:
According to the American Heart Association, risk factors for aneurysms include the following:
Risk factors for atherosclerosis, which include smoking, high cholesterol, and diabetes, may also increase the risk of fusiform aneurysm.
Aneurysms do not always cause symptoms. However, fusiform aneurysms are more likely to be symptomatic than saccular types.
Fusiform aneurysms usually occur in the brain and may cause the following symptoms:
A brain aneurysm may rupture and cause bleeding in the brain. This is a medical emergency and may lead to severe complications, including permanent brain damage and death.
A ruptured brain aneurysm may cause the following symptoms:
Find out what can cause an aneurysm to rupture.
A doctor may diagnose a fusiform aneurysm using the following methods:
If a doctor considers the risk of rupture to be low, they may want to actively observe the aneurysm through regular imaging scans. If necessary, they may suggest lifestyle changes, such as maintaining a moderate weight and stopping smoking, and prescribe medication to lower blood pressure.
If a doctor believes preventive treatment is necessary, they may recommend surgical treatment.
Doctors may find it more difficult to treat a fusiform aneurysm than a saccular aneurysm.
Treatment for an aneurysm often involves:
However, as a fusiform aneurysm balloons the artery from all sides, rather than causing a bulge on one side, as with saccular types, it may be difficult for a doctor to isolate it from the artery.
Doctors may use more advanced surgical techniques, which may include:
A person will require emergency medical treatment if a fusiform aneurysm ruptures. A doctor will usually administer nimodipine (Nymalize), a calcium channel blocker, to help reduce the risk of blood supply disruption to the brain.
Surgeons will try to repair the ruptured aneurysm with clipping or coiling procedures.
Read more about treatment for a brain aneurysm.
A person should contact a doctor if they experience symptoms of a fusiform aneurysm. Active monitoring or preventive treatment can lower the risk of rupture and other complications.
A ruptured aneurysm is a medical emergency that can lead to severe complications and death. A person should seek emergency medical attention immediately if they or someone else experiences symptoms of a ruptured aneurysm.
A person can live with an unruptured aneurysm without symptoms or complications. However, a ruptured aneurysm may cause severe health complications and death.
Fusiform aneurysms may be more challenging for doctors to treat. Experts also associate this type of aneurysm with a higher risk of complications, such as rebleeding. Rebleeding is a second rupture that occurs before doctors have treated the initial rupture, which may cause further damage or death.
According to the Brain Aneurysm Foundation, 50% to 80% of brain aneurysms remain unruptured.
Aneurysms that rupture result in death for 25% of people within 24 hours and 50% of people within 3 months.
What is the most common location for a fusiform aneurysm?Fusiform aneurysms usually occur in the brain.
How serious is a fusiform aneurysm?The severity of a fusiform aneurysm can depend on several factors, including its size, location, and risk of rupture. Fusiform aneurysms can present a higher risk of complication than saccular aneurysms.
What is a saccular aneurysm?A saccular aneurysm is a rounded, bulging sac on one side of an artery, which resembles a berry hanging from a branch.
A fusiform aneurysm causes the artery to bulge on all sides rather than ballooning on one side of the artery like a saccular aneurysm.
Doctors may find fusiform aneurysms more challenging to treat than saccular aneurysms due to their shape. Surgeons may need to use more advanced surgical techniques to seal the aneurysms, such as flow diversion or bypass procedures.
Fusiform aneurysms may present a higher risk of complications, such as rebleeding.
A person should contact a doctor if they have symptoms of an aneurysm and should seek immediate emergency medical care if they have symptoms of a ruptured aneurysm.
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