eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult
Aortic Coarctation: Treatment & Medication
Updated: Oct 2, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Severe coarctation of the aorta
- Neonates with severe coarctation of the aorta should first have their condition stabilized.
- First, support respiratory collapse with intubation. Second, infuse prostaglandin E1 to open the ductus arteriosus. Third, correct acidosis. Finally, provide inotropic support to improve symptoms of congestive heart failure.
- Less severe coarctation of the aorta
- Patients presenting with less severe coarctation of the aorta beyond the neonatal period usually have chronically increased afterload and show signs of congestive heart failure. These patients should be treated with digoxin and diuretics.
- Attempts should be made to postpone intervention, such as surgery or balloon dilatation, until the patient is hemodynamically stable.
Surgical Care
No single technique is superior to others in minimizing the rate of restenosis. The preferred method depends on anatomy of the lesion and institutional experience.- Indication for intervention: At present, 3 specific indications exist for intervention in patients with coarctation of the aorta.
- Significant coarctation or recoarctation of the aorta with long-standing hypertension with or without symptoms
- Hemodynamically significant aortic stenosis
- Female patient contemplating pregnancy
- Types of surgery
- In 1944, Blalock and Park performed the first experimental surgical repair of coarctation of the aorta in animals, which involved use of the left common carotid or subclavian artery to bypass the coarctation with end-to-end anastomosis.
- Resection of the coarctation site and end-to-end anastomosis to repair coarctation was performed first on humans in 1944 by Crafoord, Nylin, Gross, and Hufagel. This is the preferred surgical method even today. In this technique, the aorta is cross-clamped above and below the obstruction, and the discrete narrowing is resected. The advantage of this procedure is that the obstructed site is completely resected. It also avoids the use of prosthetic material and maintains a functioning left subclavian artery. The disadvantages of this procedure involve the sacrifice of spinal and intercostal vessels resulting in paralysis. Also, a high rate of restenosis exists with use of continuous running suture or circumferential fibrosis. This problem is overcome by use of interrupted and absorbable sutures, which allows for improved growth of the anastomotic site.
- Patch aortoplasty was first performed by Vossschulte in 1961 to repair coarctation of the aorta.2 This technique involves cutting across the obstruction and augmenting the area with a patch of prosthetic material. The advantages of this procedure include the ability to repair a long segment of coarctation; sparing of the left subclavian, intercostal, and spinal arteries; and preserving native aortic tissue to allow for growth. The disadvantage of this procedure is that it uses prosthetic material, which may gradually result in aneurysm formation.
- Left subclavian flap angioplasty, introduced in 1966 by Waldhausen and Nahrwold, involves ligating the left subclavian artery and dividing it distally.3 A longitudinal incision is made from the descending aorta to the coarctation superiorly into the origin of the left subclavian artery. The subclavian artery is turned down and used to enlarge the narrowing. To prevent subclavian steal phenomena, the vertebral artery is ligated. The advantages to this procedure include preservation of native vascular tissue and avoidance of circumferential sutures, which allows for better growth of the involved area. The disadvantage to this procedure is the sacrifice of a major artery to the left arm, resulting in poor growth of that extremity.
- Bypass graft repair bridges the ascending and descending aorta. The major disadvantage of this procedure is that prosthetic material does not grow as the child grows, and it becomes calcified and narrow with time.
Endovascular Care
Catheter-based intervention is now the preferred therapy for recurrent coarctation when the anatomy permits and necessary skills are available. Its use in native or unoperated coarctation is less well established. Treatment may be with balloon angioplasty alone or with a stent. Outcomes are good in skilled hands, but residual or recurrent coarctation with resultant hypertension and repair site aneurysms can occur. Catheter-based treatment can cause death from aortic rupture and dissection, but mortality compares favorably with surgery if coarctation is recurrent, and perhaps for initial treatment.
Endovascular Versus Surgical Care
The immediate improvement in hypertension and morbidity were similar across all groups. Surgical therapy was associated with a low risk of restenosis and recurrence, whereas endovascular therapy had much higher incidence of restenosis and need for repeat interventions.4 Endovascular therapy is highly promising in elderly and frail patients with multiple comorbidities who pose a high surgical risk. Overall, long-term outcome of endovascular approaches need to be evaluated.
Consultations
See Surgical Care.
Activity
As with all aortopathies and aortic valve problems, significant and prolonged isometric activities are contraindicated. The risk of dissection, even in repaired coarctation, remains significant and may be increased with isometric activity.
Medication
No specific medications are used to treat coarctation of the aorta because it is a mechanical obstruction. In the neonate, management of concomitant congestive heart failure may include prostaglandin E1 to maintain patency of the ductus arteriosus. Beyond the neonatal period, management of congestive heart failure may include digoxin and diuretics.
Prostaglandins
This agent promotes vasodilatation by direct effect on the vasculature and smooth muscle of the ductus arteriosus.
Alprostadil; Prostaglandin E1 (Prostin VR)
Used to maintain patency of ductus arteriosus when cyanotic lesion or interrupted aortic arch presents in newborn. Most effective in premature infants.
Adult
Currently used as part of protocols; recommended dosages have yet to be established
Pediatric
For palliation: 0.05-0.1 mcg/kg/min continuous IV infusion; may be increased to 0.2 mcg/kg/min if necessary
None reported
Documented hypersensitivity; respiratory distress syndrome; persistent fetal circulation
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Because of potential risk of apnea, neonates are usually intubated prophylactically; caution in neonates with bleeding tendencies; prolonged use occasionally necessary (eg, in patients with hypoplastic left heart syndrome who are transplant candidates) and may be associated with third spacing of fluid
Cardiac glycosides
These agents increase the contractility of cardiac muscle in a dose-dependent manner (ie, positive inotropic effect).
Digoxin (Lanoxin)
Cardiac glycoside with direct inotropic effects in addition to indirect effects on cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
Loading dose: 1 mg (0.5 mg then 0.25 mg q6h X 2) PO/IV
Maintenance dose: 0.125-0.375 mg/d PO/IV
Pediatric
Dosing is age and weight based; total digitalizing dose (TDD) is administered in mcg/kg/d
Loading dose: 1/2 TDD followed by 1/4 TDD q8h X 2
Maintenance dose <10 years: Divided dose bid
Maintenance dose >10 years: Administered qd
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; carotid sinus syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia may reduce positive inotropic effect of digitalis; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis
Loop diuretics
These agents inhibit electrolyte reabsorption in the thick ascending limb of the loop of Henle, thus promoting diuresis.
Furosemide (Lasix)
Commonly used diuretic with moderate diuretic potency.
Adult
Usual dose: 20-80 mg PO/IV q6-12h; not to exceed 600 mg/d
Continuous IV infusion: 0.05 mg/kg/h; titrate to effect
Pediatric
Dosages are age and weight based
Usual dose: 0.5-2 mg/kg/dose PO/IV q6-12h
Maximum dose: 6 mg/kg/dose PO; 2 mg/kg/dose IV
Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; increases auditory toxicity associated with aminoglycosides—hearing loss of varying degrees may occur; may increase anticoagulant activity of warfarin; may increase plasma lithium levels and toxicity
Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform frequent serum electrolyte, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
More on Aortic Coarctation |
| Overview: Aortic Coarctation |
| Differential Diagnoses & Workup: Aortic Coarctation |
 Treatment & Medication: Aortic Coarctation |
| Follow-up: Aortic Coarctation |
| References |
| « Previous Page | Next Page » |
References
Weinstein BM, Stemple DL, Driever W, Fishman MC. Gridlock, a localized heritable vascular patterning defect in the zebrafish. Nat Med. Nov 1995;1(11):1143-7. [Medline].
VOSSSCHULTE K. Surgical correction of coarctation of the aorta by an "isthmusplastic" operation. Thorax. Dec 1961;16:338-45. [Medline].
Waldhausen JA, Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg. Apr 1966;51(4):532-3. [Medline].
Carr JA. The results of catheter-based therapy compared with surgical repair of adult aortic coarctation. J Am Coll Cardiol. Mar 21 2006;47(6):1101-7. [Medline].
Abbruzzese PA, Aidala E. Aortic coarctation: an overview. J Cardiovasc Med (Hagerstown). Feb 2007;8(2):123-8. [Medline].
Attenhofer Jost CH, Schaff HV, Connolly HM. Spectrum of reoperations after repair of aortic coarctation: importance of an individualized approach because of coexistent cardiovascular disease. Mayo Clin Proc. Jul 2002;77(7):646-53. [Medline].
Blalock A, Park EA. The Surgical Treatment of Experimental Coarctation (Atresia) of the Aorta. Ann Surg. Mar 1944;119(3):445-56. [Medline].
Butera G, Piazza L, Chessa M, Negura DG, Rosti L, Abella R, et al. Covered stents in patients with complex aortic coarctations. Am Heart J. Oct 2007;154(4):795-800. [Medline].
Cardiac CT & MRI. www.ctcardia.com. Available at www.ctcardiac.com. Accessed April 11, 2005.
Celermajer DS, Greaves K. Survivors of coarctation repair: fixed but not cured. Heart. Aug 2002;88(2):113-4. [Medline].
Connolly HM. Pregnancy in women with coarctation of the thoracic aorta. ACC Curr J Rev. 1997;55:6-7.
Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J Thorac Surg. 1945;14:347-61.
Fawzy ME, Awad M, Hassan W, et al. Long-term outcome (up to 15 years) of balloon angioplasty of discrete native coarctation of the aorta in adolescents and adults. J Am Coll Cardiol. Mar 17 2004;43(6):1062-7. [Medline].
Golden AB, Hellenbrand WE. Coarctation of the aorta: stenting in children and adults. Catheter Cardiovasc Interv. Feb 1 2007;69(2):289-99. [Medline].
Harlan JL, Doty DB, Brandt B 3rd. Coarctation of the aorta in infants. J Thorac Cardiovasc Surg. Dec 1984;88(6):1012-9. [Medline].
Hornung TS, Benson LN, McLaughlin PR. Interventions for aortic coarctation. Cardiol Rev. May-Jun 2002;10(3):139-48. [Medline].
Karl TR. Surgery is the best treatment for primary coarctation in the majority of cases. J Cardiovasc Med (Hagerstown). Jan 2007;8(1):50-6. [Medline].
Konen E, Merchant N, Provost Y, et al. Coarctation of the aorta before and after correction: the role of cardiovascular MRI. AJR Am J Roentgenol. May 2004;182(5):1333-9. [Medline].
Konen E, Merchant N, Provost Y, McLaughlin PR, Crossin J, Paul NS. Coarctation of the aorta before and after correction: the role of cardiovascular MRI. AJR Am J Roentgenol. May 2004;182(5):1333-9. [Medline].
Perloff JK. The Clinical Recognition of Congenital Heart Disease. 3rd ed. Philadelphia, Pa: WB Saunders and Co; 1987:125-160.
Ramnarine I. Role of surgery in the management of the adult patient with coarctation of the aorta. Postgrad Med J. Apr 2005;81(954):243-7. [Medline].
Rothman A. Coarctation of the aorta: an update. Curr Probl Pediatr. Feb 1998;28(2):33-60. [Medline].
Toro-Salazar OH, Steinberger J, Thomas W, et al. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol. Mar 1 2002;89(5):541-7. [Medline].
Varma C, McLaughlin PR, Hermiller JB, Tavel ME. Coarctation of the aorta in an adult: problems of diagnosis and management. Chest. May 2003;123(5):1749-52. [Medline].
von Schulthess GK, Higashino SM, Higgins SS, et al. Coarctation of the aorta: MR imaging. Radiology. Feb 1986;158(2):469-74. [Medline].
Webb G. Treatment of coarctation and late complications in the adult. Semin Thorac Cardiovasc Surg. Summer 2005;17(2):139-42. [Medline].
Further Reading
Keywords
aorta coarctation, coarctation of aorta, cardiac lesion, juxtaductal coarctation, preductal coarctation, postductal coarctation, vascular malformation, coarctated aortic segment, infective endarteritis, cystic medial necrosis, coarctation of the aorta, narrowing of the aorta, ductus tissue theory, hemodynamic theory, ventricular septal defect, bicuspid aortic valve, left ventricular outflow obstruction, tubular hypoplasia of the transverse aortic arch, congenital cardiac lesions, XO Turner syndrome, aortic arch hypoplasia, extracardiac vascular anomalies
