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IVCS presents with a wide variety of signs and symptoms, making it difficult to diagnose clinically.
- Edema of the lower extremities (peripheral edema), caused by an increase in the blood pressure in the veins.
- Tachycardia. This is caused by the decreased preload, causing the heart to increase its frequency.
- In pregnant women, signs of fetal hypoxia and distress may be seen in the cardiotocography. This is caused by decreased perfusion of the uterus, resulting in hypoxemia of the fetus.
- Supine hypotensive syndrome
Inferior vena cava syndrome (IVCS) is a result of obstruction of the inferior vena cava. It can be caused by invasion or compression by a pathological process or by thrombosis in the vein itself. It can also occur during pregnancy.Pregnancy can lead to problems with blood return due to high venous pressure in the lower limbs, failure of blood return to the heart, decreased cardiac output due to obstructions in inferior vena cava, sudden rise in venous pressure which can lead to placental separation, and a decrease in renal function. All of these issues can arise from lying in the supine position during late pregnancy which can cause compression of the inferior vena cava. Symptoms of late pregnancy inferior vena cava syndrome consist of intense pain in the right hand side, muscle twitching, drop of blood pressure, and fluid retention.
The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitar. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration.Incidence is around 1 per 100,000 births.
Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium). This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as "Scimitar syndrome" after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a scimitar, a type of curved sword. The syndrome was first described by Catherine Neill in 1960.
Congenital stenosis of vena cava is a congenital anomaly in which the superior vena cava or inferior vena cava has an aberrant interruption or coarctation.
In some cases, it can be asymptomatic, and in other cases it can lead to fluid accumulation and cardiopulmonary collapse.
The nutcracker syndrome (NCS) results most commonly from the compression of the left renal vein between the abdominal aorta (AA) and superior mesenteric artery (SMA), although other variants exist. The name derives from the fact that, in the sagittal plane and/or transverse plane, the SMA and AA (with some imagination) appear to be a nutcracker crushing a nut (the renal vein).
There is a wide spectrum of clinical presentations and diagnostic criteria are not well defined, which frequently results in delayed or incorrect diagnosis.
This condition is not to be confused with superior mesenteric artery syndrome, which is the compression of the third portion of the duodenum by the SMA and the AA.
Penile Artery Shunt Syndrome (PASS) was initially described in a patient who underwent Penile Revascularization Surgery for isolated left cavernosal artery stenosis in the absence of systemic vascular risk factors. An end-to-end anastomosis of the left inferior epigastric artery to the left dorsal penile artery was created using a previously described technique. After technically successful revascularization surgery, the patient continued to have post-operative erectile dysfunction despite documented patency of the surgical graft by Penile Duplex Ultrasonography. Pelvic angiography was performed on the patient, revealing an aberrant obturator artery originating from the inferior epigastric artery. The study revealed markedly sluggish forward flow visualized through the anastomosis to the left dorsal penile artery, with dominant flow in the left inferior epigastric artery to the obturator artery and its branches. This culminated in reduced blood flow to the penis.
Arterial embolism can cause occlusion in any part of the body. It is a major cause of infarction, tissue death due to the blockage of blood supply.
An embolus lodging in the brain from either the heart or a carotid artery will most likely be the cause of a stroke due to ischemia.
An arterial embolus might originate in the heart (from a thrombus in the left atrium, following atrial fibrillation or be a septic embolus resulting from endocarditis). Emboli of cardiac origin are frequently encountered in clinical practice. Thrombus formation within the atrium occurs mainly in patients with mitral valve disease, and especially in those with mitral valve stenosis (narrowing), with atrial fibrillation (AF). In the absence of AF, pure mitral regurgitation has a low incidence of thromboembolism.
The risk of emboli forming in AF depends on other risk factors such as age, hypertension, diabetes, recent heart failure, or previous stroke.
Thrombus formation can also take place within the ventricles, and it occurs in approximately 30% of anterior-wall myocardial infarctions, compared with only 5% of inferior ones. Some other risk factors are poor ejection fraction (<35%), size of infarct, and the presence of AF. In the first three months after infarction, left-ventricle aneurysms have a 10% risk of emboli forming.
Patients with prosthetic valves also carry a significant increase in risk of thromboembolism. Risk varies, based on the valve type (bioprosthetic or mechanical); the position (mitral or aortic); and the presence of other factors such as AF, left-ventricular dysfunction, and previous emboli.
Emboli often have more serious consequences when they occur in the so-called "end circulation": areas of the body that have no redundant blood supply, such as the brain and heart.
Pulmonary vein stenosis is a rare cardiovascular disorder. It is recognized as being the stenosis of one or more of the four pulmonary veins that return blood from the lungs to the left atrium of the heart. In congenital cases, it is associated with poor prognosis and high mortality rate. In some people, pulmonary vein stenosis occurs after pulmonary vein ablation for the treatment of atrial fibrillation. Some recent research has indicated that it may be genetically linked in congenital cases.
Caput medusae, also known as palm tree sign, is the appearance of distended and engorged superficial epigastric veins, which are seen radiating from the umbilicus across the abdomen. The name "caput medusae" (Latin for "head of Medusa") originates from the apparent similarity to Medusa's head, which had venomous snakes in place of hair. It is also a symptom of portal hypertension. It is caused by dilation of the paraumbilical veins, which carries oxygenated blood from mother to fetus "in utero" and normally closes within one week of birth, becoming re-canalised due to portal hypertension caused by liver failure.
Determine the direction of flow in the veins below the umbilicus. After pushing down on the prominent vein, blood will:
- flow toward the legs -> caput medusae
- flow toward the head-> inferior vena cava obstruction.
There are different types of embolism, some of which are listed below.
Penile Artery Shunt Syndrome (PASS) is an iatrogenic clinical phenomenon first described by Tariq Hakky, Christopher Yang, Jonathan Pavlinec, Kamal Massis, and Rafael Carrion within the Sexual Medicine Program in the Department of Urology, at the University of South Florida, and Ricardo Munarriz, of Boston University School of Medicine Department of Urology in 2013. It may be a cause of refractory Erectile Dysfunction in patients who have undergone Penile Revascularization Surgery.
NCS is associated with hematuria (which can lead to anemia) and abdominal pain (classically left flank or pelvic pain).
Since the left gonadal vein drains via the left renal vein it can also result in left testicular pain in men or left lower quadrant pain in women. Nausea and vomiting can result due to compression of the splanchnic veins. An unusual manifestation of NCS includes varicocele formation and varicose veins in the lower limbs. Another clinical study has shown that nutcracker syndrome is a frequent finding in varicocele-affected patients and possibly, nutcracker syndrome should be routinely excluded as a possible cause of varicocele and pelvic congestion.
DVA can be characterized by the Caput medusae sign of veins, which drains into a larger vein. The drains will either drain into a Dural venous sinuses or into a deep ependymal vein. It appears to look like a Palm tree.
In medicine, May-Thurner syndrome (MTS), also known as the iliac vein compression syndrome, is a rare condition in which compression of the common venous outflow tract of the left lower extremity may cause discomfort, swelling, pain or blood clots, called deep venous thrombosis (DVT), in the iliofemoral vein.
The specific problem is compression of the left common iliac vein by the overlying right common iliac artery. This leads to pooling or stasis of blood, predisposing the individual to the formation of blood clots. Uncommon variations of MTS have been described, such as the right common iliac vein getting compressed by the right common iliac artery.
In the 21st century the May-Thurner syndrome definition has been expanded to a broader disease profile known as nonthrombotic iliac vein lesions (NIVL) which can involve both the right and left iliac veins as well as multiple other named venous segments. This syndrome frequently manifests as pain when the limb is dependent (hanging down the edge of a bed/chair) and/or significant swelling of the whole limb.
d vessels can present a large variety of , and/or . The effects may range from a change in blood pressure to an interruption in circulation, depending on the nature and degree of the misplacement and which vessels are involved.
Although "transposed" literally means "swapped", many types of TGV involve vessels that are in abnormal positions, while not actually being swapped with each other. The terms TGV and TGA are most commonly used in reference to dextro-TGA – in which the arteries "are" in swapped positions; however, both terms are also commonly used, though to a slightly lesser extent, in reference to levo-TGA – in which both the arteries and the ventricles are swapped; while other defects in this category are almost never referred to by either of these terms.
DVA can be diagnosed through the Cerebral venous sinus thrombosis with collateral drainage. DVA can also be found diagnosed with Sturge–Weber syndrome and can be found through leptomeningeal angiomatosis. Demyelinating disease has also been found to enlarge Medulla veins.
May-Thurner syndrome (MTS) is thought to represent between two and five percent of lower-extremity venous disorders. May-Thurner syndrome is often unrecognized; however, current estimates are that this condition is three times more common in women than in men. The classic syndrome typically presents in the second to fourth decades of life. In the 21st century in a broader disease profile, the syndrome acts as a permissive lesion and becomes symptomatic when something else happens such as, following trauma, a change in functional status such as swelling following orthopaedic joint replacement.
It is important to consider May-Thurner syndrome in patients who have no other obvious reason for hypercoagulability and who present with left lower extremity thrombosis. To rule out other causes for hypercoagulation, it may be appropriate to check the antithrombin, protein C, protein S, factor V Leiden, and prothrombin G20210A.
Venography will demonstrate the classical syndrome when causing deep venous thrombosis.
May-Thurner syndrome in the broader disease profile known as nonthrombotic iliac vein lesions (NIVLs) exists in the symptomatic ambulatory patient and these lesions are usually not seen by venography. Morphologically, intravascular ultrasound (IVUS) has emerged as the best current tool in the broader sense. Functional testing such as duplex ultrasound, venous and interstitial pressure measurement and plethysmography may sometimes be beneficial. Compression of the left common iliac vein may be seen on pelvic CT.
In dextro-Transposition of the great arteries (dextro-TGA) deoxygenated blood from the right heart is pumped immediately through the aorta and circulated to the body and the heart itself, bypassing the lungs altogether, while the left heart pumps oxygenated blood continuously back into the lungs through the pulmonary artery. In effect, two separate "circular" (parallel) circulatory systems are created. It is called a cyanotic congenital heart defect (CHD) because the newborn infant turns blue from lack of oxygen.
The most common cause of chronic venous insufficiency is reflux of the venous valves of superficial veins. This may in turn be caused by several conditions:
- Deep vein thrombosis (DVT), that is, blood clots in the deep veins. Chronic venous insufficiency caused by DVT may be described as postthrombotic syndrome.
- Superficial vein thrombosis.
- Phlebitis
- May–Thurner syndrome. This is a rare condition in which blood clots occur in the iliofemoral vein due to compression of the blood vessels in the leg. The specific problem is compression of the left common iliac vein by the overlying right common iliac artery. Many May-Thurner compressions are overlooked when there is no blood clot. More and more of them get nowadays diagnosed and treated (by stenting) due to advanced imaging techniques.
Deep and superficial vein thrombosis may in turn be caused by thrombophilia, which is an increased propensity of forming blood clots.
Arteriovenous fistula (an abnormal connection or passageway between an artery and a vein) may cause chronic venous insufficiency even with working vein valves.
An arteriovenous fistula is an abnormal connection or passageway between an artery and a vein. It may be congenital, surgically created for hemodialysis treatments, or acquired due to pathologic process, such as trauma or erosion of an arterial aneurysm.
Signs and symptoms of CVI in the leg include the following:
- Varicose veins
- Itching (pruritus)
- Hyperpigmentation
- Phlebetic lymphedema
- Chronic swelling of the legs and ankles
- Venous ulceration
CVI in the leg may cause the following:
- Venous stasis
- Ulcers.
- Stasis dermatitis, also known as varicose eczema
- Contact dermatitis. Patients with venous insufficiency have a disrupted epidermal barrier, making them more susceptible than the general population to contact sensitization and subsequent dermatitis.
- Atrophie blanche. This is an end point of a variety of conditions, appears as atrophic plaques of ivory white skin with telangiectasias. It represents late sequelae of lipodermatosclerosis where the skin has lost its nutrient blood flow.
- Lipodermatosclerosis. This is an indurated plaque in the medial malleolus.
- Malignancy. Malignant degeneration is a rare but important complication of venous disease since tumors which develop in the setting of an ulcer tend to be more aggressive.
- Pain. Pain is a feature of venous disease often overlooked and commonly undertreated.
- Anxiety.
- Depression.
- Inflammation
- Discoloration
- Skin thickening
- Cellulitis
Common signs and symptoms of DVT include pain or tenderness, swelling, warmth, redness or discoloration, and distention of surface veins, although about half of those with the condition have no symptoms. Signs and symptoms alone are not sufficiently sensitive or specific to make a diagnosis, but when considered in conjunction with known risk factors, can help determine the likelihood of DVT. In most suspected cases, DVT is ruled out after evaluation, and symptoms are more often due to other causes, such as cellulitis, Baker's cyst, musculoskeletal injury, or lymphedema. Other differential diagnoses include hematoma, tumors, venous or arterial aneurysms, and connective tissue disorders.
Phlegmasia cerulea dolens is a very large and dangerous type of DVT. It is characterized by an acute and almost total venous occlusion of the entire extremity outflow, including the iliac and femoral veins. The leg is usually painful, tinged blue in color, and swollen, which may result in venous gangrene.
The clinical presentation of CST can be varied. Both acute, fulminant disease and indolent, subacute presentations have been reported in the literature.
The most common signs of CST are related to anatomical structures affected within the cavernous sinus, notably cranial nerves III-VI, as well as symptoms resulting from impaired venous drainage from the orbit and eye.
Classic presentations are abrupt onset of unilateral periorbital edema, headache, photophobia, and bulging of the eye (proptosis).
Other common signs and symptoms include:
Ptosis, chemosis, cranial nerve palsies (III, IV, V, VI). Sixth nerve palsy is the most common. Sensory deficits of the ophthalmic and maxillary branch of the fifth nerve are common. Periorbital sensory loss and impaired corneal reflex may be noted. Papilledema, retinal hemorrhages, and decreased visual acuity and blindness may occur from venous congestion within the retina. Fever, tachycardia and sepsis may be present. Headache with nuchal rigidity may occur. Pupil may be dilated and sluggishly reactive. Infection can spread to contralateral cavernous sinus within 24–48 hours of initial presentation.