Coarctation of the AortaJo Ann Nieves, MSN, ARNP, CPN, PNP-BCNurse Practitioner, Adult Congenital Heart ProgramMiami Children’s HospitalMiami, FloridaDiana Arias, MSN, ARNP, FNP-C, CPNClinical Specialist, Cardiac Intensive Care UnitMiami Childre n’s HospitalMiami, FloridaI. EmbryologyA. Affects 5% to 8% of all newborns with congenital heart diseaseB. Occurs during the 6th to 8th week of gestation1. Cause of Coarctation of the Aorta (CoA) is unknown. Two theories (Beekman, 2008):a. Ductus Tissue Theory - Postnatal constriction of aberrant ductal tissueb. Hemodynamic Theory - Intrauterine alterations of blood flow through theaortic archII. Abnormal developmentA.Deformity of the aortic isthmus (where the ductus arteriosus joins the descending aorta) –characterized by narrowing of the proximal aorta or distal to the left subclavian artery. (Moon, 2011).1. Localized stenosis - a shelf-like infolding of the posterior aortic wall into the aorticlumen opposite, proximal and/or distal to the ductus arteriosus (Kaemmerer, 2011)2.Long hypoplastic segment- a tubular hypoplasia involving the aortic arch or the aortadistal to the origin of the left subclavian artery and the ductal area (Kaemmerer, 2011)Coarctation at aortic isthmus Illustrations reprinted from PedHeart Resource. .© Scientific Software Solutions, 2010. All rights reserved.B. Simple CoA - coarctation in the absence of other lesionsC. Complex CoA1. Include intracardiac and/or extracardiac lesions (Kaemmerer, 2011):a.Bicuspid Aortic valve – occurs in nearly 85%, the valve may be stenotic orthe annulus hypoplasticb.Ventricular septal defectc.Transposition of the great arteriesd.Noncardiac anomaly- aneurysm of circle of Willis in 3% to 5%D. Genetic component1. Turner XO syndrome- 35% of patients affectedIII. PhysiologyA.Left ventricular hypertension- narrowing of the aorta causes increased resistance to leftventricular outflow therefore elevating systolic pressure1. Upper extremity hypertension2. Lower extremity BP lower than the upper body BP3. “Gradient” is the difference between higher upper body & decreased lower body BP4. Fully oxygenated arterial blood – unless other lesions are presenta.Closure of foramen ovale & ductus arteriosus after birth causes entire cardiacoutput to flow through the stenotic aortic segment (Beekman, 2008)IV. Clinical Features:A. Cardinal features (Kaemmerer, 2011)1.Upper body hypertension; weak, delayed femoral pulses; a decrease in blood pressurebetween upper and lower extremities; palpable collateral arteries over the medialaspect of the scapulae, the lateral chest wall, and between the ribs2. Thrill- suprasternal notch or neck vessels3. Heave- no displaced heart soundB. Infant1. In severe CoA of the newborn, survival depends on patency of the ductus arteroisusa.When ductus arteriosus closes (approximately 8 to 10 days of life) the patientdevelops shock & heart failure. Metabolic disturbances, hypothermia,hypoglycemia can occur as well.b.Resulting in lower body & renal hypoperfusion, renal failure and/ornecrotizing enterocolitis (Beekman, 2008)C. Child or adolescent1. Upper extremity hypertensiona. Widened pulse pressure as patient gets olderb. Variability of Right and Left Arm pressures, dependent on location of CoA inrelation to the left & right subclavian artery2. Murmursa.Grade 2/6 to 3/6 systolic ejection murmur at the upper left sternal border, atthe base & left interscapular space (Beekman, 2008)D. Adults1.Patients typically diagnosed & treated earlier in life, but may rarely present withupper extremity hypertension as an adult with a native CoA (Daniels, 2008)V. Medical/surgical interventionsA. Diagnosis: most often via clinical exam, echocardiogram, and chest x-ray, MRI or CT1.Diagnostic cardiac catheterization (Beekman, 2008)a.With complex anatomy and hemodynamics, of associated lesionsb. With additional clinical questionsB. Treatment & Timing: Individualized to lesion, associated conditions1.Infant:a.If severe: signs occur in first hours of life(1) Immediate intervention required(2)Medical – initial stabilization, inotropic support(3)Prostaglandin E1 IVMaintain open ductus arteriosusAllows for flow from RV to enter MPA, cross the ductus,enter the aorta, and perfuse the descending aorta, renal andmesenteric arteriesb. Surgical CoA repair(1) Individualized to anatomy of CoA(2)Plan to include treatment of any additional cardiac defects2.Child, Adolescenta.Repair at 2 to 3 years of ageb.Upon diagnosisC. Surgery -4 Common types of repair- regardless of technique, usually performed via a left thoracotomy incision1. End-to-end anastomosis– 1954 by Crawford and Nylin (See illustration below)a. Surgical treatment of choice in most centersb. Excision of CoA area, circumferential anastomosis is completed withinterrupted sutures anteriorly (Beekman)Ductal SegmentEnd-to-End Anastomosis with removal of Ductal SegmentIllustrations reprinted from PedHeart Resource. .© Scientific Software Solutions, 2010. All rights reserved.2. Left subclavian flap – 1966 by Waldhausen and Nahrwold (Beekman, 2008)a. Ligate left subclavian artery, open the proximal subclavian artery andbeyond the CoAb. Fold subclavian artery flap down over the CoA section and sutureinto placeSubclavian arteryFlapCoarctation Repair with Left Subclavian FlapIllustrations reprinted from PedHeart Resource. .© Scientific Software Solutions, 2010. All rights reserved.3. Prosthetic patch aortoplasty- 1961 by Vosschulte (Beekman, 2008)a. Longitudinal incision is made across the CoAb. Area enlarged with a Dacron or Gore-tex patch4. Bypass graft – a tube is sewn in between ascending and descending thoracic aortaD. Outcomes1. Mortality rates vary on patient age and associated lesions (Kaemmerer, 2011)a. Simple CoA- Less than 1% mortalityb. Age 2 to 5- best age to electively operate due to low surgical riskc. Death rates strongly related to complexity of lesionsd. After age 30 or 40- intraoperative mortality rate increases due to degenerativechanges to the aortic wall.2. Morbiditya. Post op –potential paradoxical hypertension, spinal cord ischemia & paralysis,recurrent laryngeal or phrenic nerve injury, chylothorax, bleeding, infectionE. Significant long term issues: See Section VII BVI. Cardiac Catheterization: Interventional (Beekman, 2008)A. Balloon angioplasty began 1982,1. Initially accepted for treatment of re-coarctaton2. Not completely accepted for treating primary CoA3. Balloon Angioplasty – enlarges CoA lumen, produces linear intimal andmedial tears at the CoA siteBalloon Angioplasty with Implantation of StentIllustrations reprinted from PedHeart Resource. .© Scientific Software Solutions, 2010. All rights reserved.4. Risk: Aneurysm from extension to adventitiaB. Stent implantation following CoA angioplasty (See illustration above)1. Endovascular buttress, supports dilated aortic segment2. Restenosis uncommon3. Redilatation may be needed as child growsC. Outcomes:1. Mortality- rare beyond newborn perioda. Higher rate has been reported for angioplasty for recurrent post op CoA versusnative CoA2. Acute complications (Beekman, 2008)a. Femoral artery injury and thrombosis- common in infants younger than 12monthsb. Femoral artery hemorrhagec. Cerebrovascular accident3.Long-term problemsa.Coronary artery stenosisb.Dilation of neo-aortic rootc.Aortic valve regurgitationVII. Long TermA. Excellent prognosis for normal growth, development when lesion successfullyrepaired in childhood (Beekman, 2008)B. Potential complications and risks1. May occur after all forms of repairs (Kaemmerer, 2011)2. Residual CoA – presence of gradient in aorta after repair3. Recurrent CoA– development of restenosis, gradient in aorta after an initiallysuccessful repaira. 8% to 54% (Daniels, 2008)b. Recoarctation- aortic isthmus and/or aortic archd.May cause systemic hypertension, heart failure, left ventricular wall mass,coronary artery disease4. Systemic arterial hypertensiona. At rest or during exercise (Kaemmerer, 2011)b. Related to re-coarctation, structural changes in the wall of vessels, reducedbaroreceptor sensitivity, changes in renin-angiotensin system, elevatedplasma levels of epinephrine and norepinephrine, or coexistence of essential hypertensionc. Higher risk of residual hypertension & early atherosclerotic disease if repairsoccur late in childhood and adolescence (Maron, 1973)d. Systolic hypertension may occur during dynamic exercise (Beekman, 2008)5. Coronary artery disease6. Progression valve disease, bicuspid aortic valve or mitral valve (Daniels, 2008a)a. Bicuspid aortic valve can progress to stenosis (59-81%) or regurgitation (13-22%) (Sabet, 1999)7. Aortic aneurysm at the site of CoA, ascending or descending aortaa. Highest after prosthetic patch aortoplastyb. Risk rupture (Kaemmerer, 2011) – dangerous, life threatening8. Other vascular problems: Aortic dissection, intracranial hemorrhage (may berelated to berry aneurysms in circle of Willis) (Beekman, 2008)9. Long term concerns may be greatly affected by associated cardiac lesions10. Left shoulder elevation- seen in adults due to left lateral thoracotomy11. Left arm – decreased pulse/ BP if surgery used left subclavian artery patch12. Sudden death (Daniels, 2008)13. Bacterial endocarditisa. Antibiotic prophylaxis prior to dental procedures no longer required byAmerican Heart Association, 2007b. Should seek additional information regarding status of other lesionsVIII. Adults with CoA repair: Routine cardiology careA. Annual visits: Complex congenital heart disease classification (Kaemmerer, 2011)1. Clinical evaluationa. Documentation of type of CoA repair is importantb. Monitor blood pressuresc. Assessment NOTE: Using four extremities BP needed(1)Monitor for upper body hypertension, weak femoral pulses, gradientbetween upper and lower body BP(2)If left subclavian artery u sed as part of repair, BP’s will be LOWERin the left arm (avoid use of arterial line here)(3)If aberrant subclavian artery present – must consider use of left armto obtain a BP which is proximal to CoA repaird. Electrocardiograme. Transthoracic echof. Cardiac magnetic resonance (MRI) or CT(1)Surveillance aorta for potential aneurysms, status of aortic repair,valvesg. Exercise test: Surveillance for exercise induced hypertensionh. Monitor new or different type headache(1) May be sign of possible cerebral aneurysmi. Monitor cholesterol; avoid obesity & smoking(1) Minimize additional risk for coronary artery disease)j. Education(1)Assess knowledge, review condition, life long care needs(Resources: American Heart Association (),Adult Congenital Heart Association ()(2)Pregnancy information website:/B. Care during pregnancy (Refer to problem section on pregnancy in adults with CHD1. Recommendationsa. Consultation: Adult congenital heart cardiologist before pregnancyb. Multidisciplinary observation by experienced cardiologist during pregnancy, labor, delivery and for period of time post-partumc. Highest risks(1)Unrepaired CoA, arterial hypertension, residual CoA, aneurysm(Kaemmerer, 2011)References:Beekman RH. Coarctation of the Aorta. In Allen HD, Clark EB, Gutgesell HP, Driscoll D.J. Moss and Adams’ Heart Disease in Infants, Children’ and Adolescents, 6th Ed, Philadelphia, 2001, Lippincott Williams & Wilkins (p. 987-1005).Bromberg BI, Beekman RH, Rocchini AP, et al. Aortic aneurysm after patch aortoplasty repair of coarctation: A prospective analysis of prevalence, screening tests and risks. J Am Coll Cardiol 14:734-741, 1989.Daniesl CJ. The Adolesent and adult with congenial heart disease. In Allen HD, Clark EB, Gutgesell HP, Driscoll D.J. Moss and Adams’ Heart Disease in Infants, Children’ and Adolescents, 6th Ed, Philadelphia, 2008, Lippincott Williams & Wilkins (p. 1370-1397).Daniesl CJ. (a) Adult congenial heart disease: Lesion specific pathways. In Allen HD, Clark EB, Gutgesell HP, Driscoll D.J. Moss and Adams’ Heart Disease in Infants, Children’ and Adolescents, 6th Ed, Philadelphia, 2008a, Lippincott Williams & Wilkins (p. 1427-1430).Illustrations reprinted from PedHeart Resource. . © ScientificSoftware Solutions, 2010. All rights reserved.Kaemmerer H. Aortic coarctation and interrupted aortic arch. In Gatzoulis MA, Webb GD, Daubeney PEF. Diagnosis and Management of Adult congenital Heart Disease, 2nd Edition. Philadelphia , 2011, Elsevier (p. 261-270).Maron BJ, Humphries JO, Rowe RD, et al. Prognosis of surgically corrected coarctation of the aorta: A 20 year post-operative appraisal. Circulation 47:119-126, 1973.Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally bicuspid aortic valves: A surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2715 additional cases. Mayo Clinic Proc 74:14-26, 1999.8/2011Below is given annual work summary, do not need friends can download after editor deleted Welcome to visit againXXXX annual work summaryDear every leader, colleagues:Look back end of XXXX, XXXX years of work, have the joy of success in your work, have a collaboration with colleagues, working hard, also have disappointed when encountered difficulties and setbacks. Imperceptible in tense and orderly to be over a year, a year, under the loving care and guidance of the leadership of the company, under the support and help of colleagues, through their own efforts, various aspects have made certain progress, better to complete the job. For better work, sum up experience and lessons, will now work a brief summary.To continuously strengthen learning, improve their comprehensive quality. With good comprehensive quality is the precondition of completes the labor of duty and conditions. A year always put learning in the important position, trying to improve their comprehensive quality. Continuous learning professional skills, learn from surrounding colleagues with rich work experience, equip themselves with knowledge, the expanded aspect of knowledge, efforts to improve their comprehensive quality.The second Do best, strictly perform their responsibilities. Set up the company, to maximize the customer to the satisfaction of the company's products, do a good job in technical services and product promotion to the company. And collected on the properties of the products of the company, in order to make improvement in time, make the products better meet the using demand of the scene.Three to learn to be good at communication, coordinating assistance. On‐site technical service personnel should not only have strong professional technology, should also have good communication ability, a lot of a product due to improper operation to appear problem, but often not customers reflect the quality of no, so this time we need to find out the crux, and customer communication, standardized operation, to avoid customer's mistrust of the products and even the damage of the company's image. Some experiences in the past work, mentality is very important in the work, work to have passion, keep the smile of sunshine, can close the distance between people, easy to communicate with the customer. Do better in the daily work to communicate with customers and achieve customer satisfaction, excellent technical service every time, on behalf of the customer on our products much a understanding and trust.Fourth, we need to continue to learn professional knowledge, do practical grasp skilled operation. Over the past year, through continuous learning and fumble, studied the gas generation, collection and methods, gradually familiar with and master the company introduced the working principle, operation method of gas machine. With the help of the department leaders and colleagues, familiar with and master the launch of the division principle, debugging method of the control system, and to wuhan Chen Guchong garbage power plant of gas machine control system transformation, learn to debug, accumulated some experience. All in all, over the past year, did some work, have also made some achievements, but the results can only represent the past, there are some problems to work, can't meet the higher requirements. In the future work, I must develop the oneself advantage, lack of correct, foster strengths and circumvent weaknesses, for greater achievements. Looking forward to XXXX years of work, I'll be more efforts, constant progress in their jobs, make greater achievements. Every year I have progress, the growth of believe will get greater returns, I will my biggest contribution to the development of the company, believe inyourself do better next year!I wish you all work study progress in the year to come.。
