The age of onset of hypertension is getting younger and younger. In addition to and lifestyle and other factors, you need to pay attention to whether hypertension is caused by other diseases and discover the cause, hypertension may be cured!
Fibromuscular dysplasia is a non-atherosclerotic, non-inflammatory vascular disease that can lead to arterial stenosis, occlusion, hemangioma or entrapment. It most often involves the renal arteries and extracranial carotid and vertebral arteries. The clinical presentation varies depending on the vessels involved: involvement of the renal arteries most commonly presents with hypertension, while involvement of the carotid or vertebral arteries may cause dizziness, throbbing tinnitus, transient ischemic attack or stroke.
Fibromuscular dysplasia is often chronically underdiagnosed or misdiagnosed in clinical practice, with an average delay of 4 to 9 years from first signs and symptoms to diagnosis, which can be attributed to a variety of reasons: many clinicians know little about the disease and often do not consider it as a differential diagnosis; many of the presentations of the disease lack specificity. Delays in diagnosis can lead to impaired quality of life and poor prognosis, such as poorly controlled hypertension, development of hypertensive complications, TIA, stroke, entrapment, or aneurysm rupture.
Fibromuscular dysplasia is poorly recognized clinically, but the incidence is not low, with an estimated 4 cases per 100 adults.
Historical review
The first case was reported in 1938, a 5½-year-old boy with severe hypertension and partial obstruction of the renal artery, which was cured by postoperative hypertension.
In 1958, fibromuscular dysplasia was named.
In 1961, several authors summarized its clinical presentation and arteriographic manifestations, which led to a more widespread recognition of the disease.
In 1965, investigators reported the first histologically confirmed case of fibromuscular dysplasia of the internal carotid artery. This was a case of a 34-year-old woman with cerebral thrombosis resulting in right-sided hemiparesis and aphasia.
In 1967, a woman with bilateral internal carotid artery fibromuscular dysplasia had transient ischemic symptoms that resolved after surgery.
In 1971, investigators proposed a detailed pathologic staging (and associated angiographic presentation) of fibromuscular dysplasia of the renal arteries.
In 2012, the American Registry of Fibromuscular Dysplasia published information on the first 447 patients.
In 2014, the American Heart Federation published a scientific statement for this disease.
Clinical presentation
The clinical manifestations of fibromyalgia are varied and depend on a number of factors, the most important of which are the distribution of the affected vascular bed and the type and severity of the vascular lesion (i.e., degree of stenosis, arterial entrapment, aneurysm). Among the clinical manifestations: hypertension 63.8%, headache 52.4%, pulsatile tinnitus 27.5%, dizziness 26%, cervical vascular murmur 22.2%, neck pain 22.2%, chest pain or shortness of breath 16.1%, (hypertensive) abdominal pain 15.7%, aneurysm 14.1%, carotid artery entrapment 12.1%, epigastric vascular murmur 9.4%, TIA 8.7%, postprandial abdominal pain 7.8%, stroke 6.9%, claudication 5.2%, black mask 5.2%, weight loss 5.2%, Horner syndrome 4.7%, renal artery entrapment 3.1%, azotemia 2%, myocardial infarction 1.8%, mesenteric ischemia 1.3%.
The most common clinical manifestation of renal artery fibromuscular dysplasia is hypertension. the development of hypertension or refractory hypertension before the age of 35 years should be considered as a possibility, however, the mean age at which hypertension occurs in this disease is 43.1 years, which has a significant overlap with the age of patients with essential hypertension.
In addition, a vascular murmur in the epigastrium or hypochondrium on physical examination is a possible manifestation of renal artery FMD. Coeliac pain may be a manifestation of renal artery entrapment or hemangioma, but is also seen in patients with uncomplicated renal artery FMD. Renal insufficiency is a rare manifestation. Renal artery entrapment and renal infarction may lead to chronic nephropathy, but FMD alone leading to end-stage renal disease is rare. Interestingly, headaches are also common in patients with isolated renal artery FMD and well-controlled blood pressure.
Diagnostic strategies for renal artery FMD
The primary method of diagnosing FMD is imaging. Noninvasive imaging includes Doppler ultrasound, CT angiography (CTA) and magnetic resonance angiography (MRA), with catheter angiography as the gold standard.IVUS and manometry can confirm the diagnosis in some ambiguous cases and also help to evaluate the hemodynamic significance of the stenosis and the effectiveness of percutaneous intervention.
Treatment of fibromuscular dysplasia Advances in imaging techniques, medical therapy, and endovascular therapy have made treatment of patients with this disease less invasive, safer, and more effective. Treatment options include: endotherapy with monitoring; endovascular treatment for stenosis (angioplasty with or without stenting), endovascular treatment for entrapment (stenting), endovascular treatment for aneurysms (spring coils, stents); and surgery. Treatment decisions depend on the characteristics and location of the vascular lesion (stenosis, entrapment, aneurysm), the presence and severity of symptoms, prior FMD-related vascular events, the presence and size of the aneurysm, and comorbidities.
Common misconceptions about fibromuscular dysplasia are poorly understood by many physicians regarding this disease, including a plethora of misconceptions about FMD that are constantly repeated in the literature.
Misconception 1.
All coronary, carotid and renal artery disease is the result of atherosclerosis.
Facts.
1) Fibromuscular dysplasia can cause renal artery, visceral artery, cerebrovascular, limb artery and coronary artery disease.
2) The mean age of diagnosis of fibromuscular dysplasia is 51.9+/-13.4 years (range 5-83 years).
3) Many patients have no or almost no atherosclerotic risk factors.
4) Atherosclerosis is commonly seen in the open or proximal segments of blood vessels and fibromuscular dysplasia is seen in the middle and distal segments of arteries.
Misconception 2.
The severity of multifocal fibromuscular dysplasia (mesofibrillar hyperplasia) can be accurately determined by direct angiography.
Facts.
1) The severity of stenosis cannot be accurately determined by angiography or other imaging techniques.
2) Patients with fibromuscular dysplasia should have their IVUS checked or pressure step differences measured before and after angioplasty.
3) Up to one third of patients without stenosis on angiography after angioplasty still have participating stenosis on IVUS or pressure step difference measurement.
Misconception 3.
Doppler ultrasound flow velocity predicts the severity of carotid or renal artery fibromuscular dysplasia.
Facts.
1) The degree of “stenosis” cannot be determined by Doppler velocity changes.
2) There are no diagnostic velocity criteria for cerebrovascular or renal artery fibromuscular dysplasia, unlike Doppler measurements for atherosclerotic carotid or renal artery disease.
3) For ultrasound reports, the following description is recommended: “Increased flow velocity (PSV, 450 cm/s), turbulence and tortuosity in the middle and distal segments of the renal artery (or carotid artery), consistent with fibromuscular dysplasia”, which is more accurate than saying the degree of arterial stenosis (e.g., 50%-70%).
Misconception 4.
Stents should be implanted for interventional treatment of fibromuscular dysplasia of the renal or carotid arteries.
Facts.
1) There is no direct indication for implantation in most cases of fibromuscular dysplasia treatment.
2) Angioplasty alone should be used to relieve the pressure gradient and correct the normal appearance of IVUS.
3) Fibromuscular dysplasia is seen in the middle and distal segments of the vessel; therefore, stenting of the renal artery in the event of restenosis increases the complexity of surgical repair.
4) The only indications for stenting are the inability to obtain a satisfactory result with PTA alone (which is rare) and treatment resulting in entrapment.
Misconception 5.
The most common presentation of carotid fibromuscular dysplasia is TIA or stroke.
Facts.
1) Although carotid fibromuscular dysplasia can present with TIA, stroke, and carotid artery entrapment, the most common presentation is nonspecific.
2) Non-specific symptoms of carotid fibromuscular dysplasia include headache, dizziness, light-headedness and pulsating tinnitus.
3) Carotid fibromuscular dysplasia can also be asymptomatic, with an unexpected finding or detection of a vascular murmur in the neck on imaging.