Flachskampf2, raimund erbel3, francesco antoninicanterin4, charalambos vlachopoulos5, guido rocchi6, rosa sicari7, petros nihoyannopoulos8, and jose zamorano9 on behalf of the european association of echocardiography. Jan 01, 2020 management of coa during pregnancy is complicated by heightened procedural risks to the pregnant woman and her fetus. Aortic coarctation with extensive collateral circulation. Cardiovascular magnetic resonance imaging can be helpful in management. A second scenario is the progression of imh to classic aortic dissection between an initial diagnostic study and an interim followup image. Although most patients have a discrete narrowing of the thoracic aorta at the insertion of the ductus arteriosus, the anatomical spectrum may vary from this discrete entity to tubular hypoplasia, with many variations in between.
Clinical, diagnostic, and management perspectives of aortic dissection ijaz a. Print version issn 01044230online version issn 1806. Full text diagnosis and clinical management of aortic. Ac accounts for 58% of all congenital heart diseases with a maletofemale ratio of 41 but its. Clinical, diagnostic, and management perspectives of. Characterized by blood pressure differential between upper and lower extremities upper lower. For aortic procedures, high estrogen levels can impact aortic remodeling, predisposing the aorta to dissection and rupture 3,6. In older children and adults, echocardiographic windows are often not adequate to fully assess the aortic vasculature. Nair, md, fccp the incidence of aortic dissection ranges from 5 to 30 cases per million people per year. The first surgical repair of aortic coarctation was described in 1944, and since that time, several other surgical techniques have been developed and modified. The aortic arch is the portion of the main artery that bends between the ascending and descending aorta. Aortic regurgitation of any degree accompanies 18 to 50% of cases with proximal aortic dissection. The vascular malformation responsible for coarctation is a defect in the vessel media, giving rise to a prominent posterior infolding the posterior shelf, which may extend around the entire circumference of the aorta. Coarctation of the aorta symptoms and causes mayo clinic.
There is no established evidence on the duration of follow up. The diagnosis of aortic dissection has been missed in up to 38% of patients on initial evaluation, and in up to 28% of patients the diagnosis has been first established at the post mortem examination. Feb 26, 2018 torosalazar oh, steinberger j, thomas w, rocchini ap, carpenter b, moller jh. Nair, md, fccp the incidence of aortic dissection ranges from 5 to 30 cases per million people per year, depending on the prevalence of risk factors in the study population. Adult coarctation endarteritis is a rare entity but often represents the initial presentation of coarctation. You or your child may be more likely to have aortic coarctation if certain heart conditions exist, including. Coarctation of aorta clinicals, diagnosis, and management. Aortic coarctation with extensive collateral circulation aha journals.
Longterm followup of patients after coarctation of the aorta repair. The word coarctation means pressing or drawing together. Diagnosis, imaging and clinical management of aortic coarctation elles j dijkema,1 tim leiner, 2 heynric b grotenhuis1 abstract coarctation of the aorta coa is a wellknown congenital heart disease chd, which is often associated with several other cardiac. Presurgical contrast enhanced ct scan demonstrating a marked narrowing of descending thoracic aorta at the level of where the ligamentum arteriosum inserts into the descending aorta and is distal to the left subclavian artery keeping with the infantilepreductal form of aortic coarctation. Therefore, keeping a high clinical index of suspicion is crucial in establishing the diagnosis of aortic dissection. Coarctation of the aorta coa or coao, also called aortic narrowing, is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus ligamentum arteriosum after regression inserts. Aortic coarctation ac is defined as a local narrowing of the aortic lumen, which is located at the level of the isthmus in 95% of patients i. Coarctation of the aorta often occurs along with other congenital heart defects, although doctors dont know what causes multiple heart defects to form together. It typically presents with upper extremity systolic hypertension or murmur.
Coarctation of the aorta coa is usually diagnosed in childhood, but 20%. The diagnosis and management of aortic dissection sri g thrumurthy, alan karthikesalingam, benjamin o patterson, peter j e holt, matt m thompson summary points aortic dissection is diagnosed and managed according to its anatomical extent and chronicity white men aged over 40 years with hypertension, or those under 40 with marfans syndrome. Acute aortic syndrome risk factors and clinical presentation there are many risk factors that predispose to the development of aas, either through weakening of the aortic media or by exposing the wall of the aorta to increased hydraulic stress table 1. Primarycare management of patients with coarctation of the. Coarctation of the aorta c is the sixth most common congenital lesion accounting for 46 per cent of live births with congenital heart disease 1,2. If you have had aortic coarctation repair and are considering becoming pregnant, careful management of your blood pressure is important to help keep you and your baby healthy. See clinical manifestations and diagnosis of coarctation of the aorta. Esc guidelines on the diagnosis and treatment of aortic diseases external link opens in a new window.
Nov 26, 2015 coarctation is a heterogeneous lesion which may present across all age ranges, with varying clinical symptoms, in isolation, or in association with other cardiac defects. Aortic coarctation produces conditions highly conducive to the development of endothelial injury and. Diagnosis, imaging and clinical management of aortic coarctation. Velocityencoded cine magnetic resonance mr imaging is becoming the modality of choice for the clinical evaluation of aortic coarctation, a congenital narrowing of the thoracic aorta, in which a functional assessment of hemodynamic obstruction is as important as anatomic delineation. Coarctation of the aorta is a common malformation 410 000 live births to be ruled out in this setting. Eight studies were performed preoperatively and six postoperatively. Coarctation of the aorta diagnosis and treatment mayo. Multimodality imaging of thoracic aortic diseases in. Management of severe coarctation of the aorta during. Prestenotic dilatation of the aortic arch and left subclavian artery, as well as indentation at the site of coarctation results in a classic figure 3 sign on xray.
Pdf preoperative physiology, imaging, and management of. Pdf the importance of the early diagnosis of aorta coarctation. Also 3dimensional 3d and 4dimensional 4d imaging with or without stic has been suggested to be used as newer techniques to improve diagnosis of coarctation in fetal life. Diagnosis and clinical management of aortic dissection phillips b harrington, james e davies, spencer j melbydivision of cardiothoracic surgery, department of surgery, university of alabama at birmingham, birmingham, al, usaabstract. Velocityencoded cine mr imaging in aortic coarctation. This defect generally results in left ventricular pressure overload. It accounts for 68% of congenital heart defect cases and ranks as the fifth most common abnormality in this patient group. Aortic coarctation symptoms, diagnosis and treatment bmj. If the narrowing is severe, it may present in the newborn period once the ductus arteriosus closes as low cardiac output and shock critical coarctation. Aug 17, 2018 cardiac magnetic resonance shows severe coarctation at the level of the aortic isthmus and extensive thoracic and abdominal collateral circulation. Diagnosis is critically important given the risk of rupture.
Choice of imaging modality for aortic dissection goldstein jase 2015 28. Feb 26, 2018 aortic coarctation is a narrowing of the aorta most commonly found just distal to the origin of the left subclavian artery. They should be essential in everyday clinical decision making. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally. Coarctation of the aorta diagnosis and treatment mayo clinic.
Peaktopeak coarctation gradient,20 mg with imaging evidence of significant coarctation and. Catheterization data were available on 12 patients for. Imaging can also provide important ancillary information regarding myocardial performance, aortic valve morphology and function, and endorgan perfusion. Coarctation of aorta coa is a discrete narrowing in aorta causing obstruction to. Pdf coarctation of the aorta c is the sixth most common lesion in. Coarctation of the aorta coa is a wellknown congenital heart disease chd, which is often associated with several other cardiac and vascular. Management of descending thoracic aorta diseases external link opens in a new window.
Noninvasive diagnosis of aortic coarctation in neonates with patent ductus arteriosus. Male infants are affected more commonly, and the incidence is likely higher in stillborn infants. Clinical manifestations and diagnosis of coarctation of the. With imaging, resorption of the lower part of the ribs may be seen, due to increased blood flow over the neurovascular bundle that runs there. Clinical, diagnostic, and management perspectives of aortic.
Endocarditis is an inflammation of the inner lining of the heart or of its structures, caused by a bacterial infection. Recommendations echocardiography in aortic diseases. Nov 16, 2011 other factors contributing to a delay in diagnosis include a lack of familiarity by clinicians with the clinical manifestations of coarctation of the aorta. It is estimated that approximately 60% to 80% of newborns with aortic coarctation are not diagnosed before hospital discharge. Eae recommendations for clinical practice arturo evangelista1, frank a. The importance of the early diagnosis of aorta coarctation. Management of acute aortic dissection and related syndromes requires a multidisciplinary approach with input from the patient, clinician, imager, surgeon, and anesthesiologist. Despite echocardiographic screening, prenatal diagnosis of c o a remains difficult. The underlying pathology of aortic coarctation, deceptively, appears simple. Diagnostic imaging studies in the setting of suspected aortic dissection have important primary goals, such as confirmation of clinical suspicion, classification of dissection, localization of tears, and assessment of both extent of dissection and indicators of emergency e. The american college of cardiology and american heart association guidelines for adults with congenital heart disease recommend intervention in patients who have a peaktopeak.
Treatment in patients with congestive heart failure chf includes the use. Diagnosis, imaging and clinical management of aortic coarctation elles j dijkema,1 tim leiner, 2 heynric b grotenhuis1 abstract coarctation of the aorta coa is a wellknown congenital heart disease chd, which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve bav. Pdf coarctation of aorta coa is a discrete narrowing in aorta causing obstruction to the flow of blood. Velocityencoded cine mr imaging may be used to guide the clinical management of aortic coarctation before, during, and after intervention. These include the presence of additional cardiovascular anomalies predominantly aortic valve abnormality and presentation with complications after coarctation repair in childhood such as recurrent coarctation or aneurysm formation. Aortic dissection is a potentially lethal clinical entity that requires rapid diagnosis, appropriate medical management, and potential surgical intervention. Coarctation of the aorta coa is a wellknown congenital heart disease chd, which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve bav, ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. It is often associated with a diffuse arteriopathy and bicuspid aortic valve. Aortic coarctation is a common congenital heart disease. Management of acute aortic dissection christoph a nienaber, rachel e clough a new appraisal of the management of acute aortic dissection is timely because of recent developments in diagnostic strategies including biomarkers and imaging, endograft design, and surgical treatment, which have led to a better.
Surgery is the preferred treatment of infants with coarctation with an overall. Torosalazar oh, steinberger j, thomas w, rocchini ap, carpenter b, moller jh. Diagnosis is made by demonstration of aortic arch narrowing, typically by echocardiog. Definition and pathology coarctation of the aorta is defined as a congenital cardiac anomaly consisting of a constricted aortic segment com. Coarctation is a heterogeneous lesion which may present across all age ranges, with varying clinical symptoms, in isolation, or in association with other cardiac defects. Although coarctation of the aorta may be repaired, youll need careful followup throughout life in order for doctors to monitor you for complications and recurrences. Catheterization data were available on 12 patients for verification of mr imaging findings. Coarctation of the aorta is a relatively common form of congenital heart disease, with an estimated incidence of approximately 3 cases per 0 births. Epidemiology coarctations account for between 58% of all congenital heart defects. Clinical signs may include upper extremity hypertension, weak. Coarctation of the aorta radiology reference article. For any given clinical scenario, the imaging modality and protocol chosen will have a significant impact on sensitivity and specificity for the aortic diagnosis of concern. The purpose of this article is to summarize the roles of ct and mri in the diagnosis and followup of patients with aortic coarctation.
In these cases, followup imaging with computed tomography angiography or contrastenhanced magnetic resonance imaging allows assessment of. Adequate and timely diagnosis of coa is crucial for good prognosis, as early treatment is associated with lower risks of longterm morbidity and mortality. Congenital heart disease aortic coarctation bicuspid aortic valve cardiac surgery stent. Other factors contributing to a delay in diagnosis include a lack of familiarity by clinicians with the clinical manifestations of coarctation of the aorta. The role of imaging in aortic dissection and related. Management of severe coarctation of the aorta during pregnancy. Current methods of intervention may involve surgical repair with the placement of a synthetic patch, percutaneous balloon dilation, or imaging guided stent placement.
Diseases of the aorta organization of professionals. The condition is more common in males than in females. The management of recurrent aortic coarctation may be challenging, especially if the recurrent segment is long or in cases of associated aortic arch hypoplasia. Echocardiography post operative echocardiogram ensure the restoration of normal anatomy. Role of surgery in the management of the adult patient. It accounts for 68% of all congenital heart diseases. The clinical manifestations, natural history, and diagnosis of coa are discussed separately. Coarctation of aorta coa is a discrete narrowing in aorta causing obstruction to the flow of blood.
However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for noninvasive diagnosis and followup of coa. However, these patients continue to require lifelong followup for management of associated problems including arterial hypertension, atherosclerotic disease, re. Physical exam may show diminished, absent, or delayed lower extremity pulses compared with upper extremity pulses. Coarctation of aorta is a life long disease and long term follow up is indicated after the definitive treatment. Esc clinical practice guidelines aim to present all the relevant evidence to help physicians weigh the benefits and risks of a particular diagnostic or therapeutic procedure on aortic diseases. Pdf current management of coarctation of the aorta researchgate. Coarctation of the aorta is a congenital heart disease characterized by a narrowing that occurs. In these cases, followup imaging with computed tomography angiography or contrastenhanced magnetic resonance imaging allows assessment of the aortic vasculature in great detail. Thirteen patients, aged 331 years, with coarctation of the thoracic aorta were examined by magnetic resonance mr imaging total of 14 studies. Diagnosis, imaging and clinical management of aortic.
Advances in the diagnosis of acute aortic syndromes. Aortic coarctation symptoms, diagnosis and treatment. Chest pain in a pregnant patient with history of coa raises concerns for aortic aneurysm with dissection, coronary artery dissection, pulmonary embolism, pericarditis, or myocardial infarction. Aortic coarctation is a common congenital heart disease accounting for approximately 68% of congenital heart defects. Aortic aneurysm and acute aortic syndrome are not uncommon conditions. Keywords acute aortic syndromes, angiography, aortic diseases, aortic dissection, ct. Clinical manifestations and diagnosis of coarctation of. Explore mayo clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease lifestyle and home remedies. Coarctation of the aorta coa is a narrowing of the descending aorta, which is typically located at the insertion of the ductus arteriosus just distal to the left subclavian artery. Coarctation of the aorta coa refers to a narrowing of the aortic lumen. Cardiac magnetic resonance shows severe coarctation at the level of the aortic isthmus and extensive thoracic and abdominal collateral circulation. Adult patients with coarctation of the aorta have a range of clinical presentations.
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