eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Coarctation of the Aorta: Follow-up

Author: P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
Coauthor(s): Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Contributor Information and Disclosures

Updated: Jul 20, 2009

Follow-up

Further Inpatient Care

  • Focus postoperative recovery of patients with coarctation of the aorta (CoA) on control of blood pressure (BP), slow advancement of diet, manipulation of antihypertensive medications, progressive ambulation, and pulmonary therapy to improve left lung atelectasis (common after left thoracotomy).
  • Hospitalization and inpatient care may be required for cardiac catheterizations, catheter interventions, and surgical procedures for treatment of recoarctation or other complications.

Further Outpatient Care

  • Focus early outpatient follow-up care on wound healing, resolution of lung atelectasis, and adjustment of antihypertensive medications.
  • Assess postoperative BP response to exercise prior to discontinuation of antihypertensive therapy.
  • Many patients may be weaned from antihypertensive therapy over months or years following repair of coarctation. Other patients may require some form of ongoing antihypertensive therapy.
  • Search for late complications of coarctation repair, including recurrent coarctation and aneurysm formation.
  • Patients should receive antibiotic prophylaxis before undergoing any bacteremia-producing surgery or procedures, especially in view of reported incidence of bacterial endocarditis during follow-up.149

Transfer

  • If coarctation of the aorta is prenatally diagnosed, the mother should deliver at or near a facility with expertise in caring for an infant with coarctation of the aorta. The facility should also have prostaglandin E1 available.
  • Infants who present early with severe coarctation of the aorta may require transfer to a facility with pediatric cardiologists and cardiovascular surgeons skilled in the management of coarctation in seriously ill infants.
  • Consider transfer of older children, adolescents, and adults to a facility with cardiologists and surgeons with experience in the management of coarctation.

Deterrence/Prevention

  • Although coarctation of the aorta cannot be prevented, it can be detected early with fetal echocardiography. Although the diagnosis can be difficult to make, echocardiography may be considered in fetuses with unexplained enlargement of the right ventricle, difficult identification of the true aortic arch, identification of an unusual aortic arch contour, or identification of anomalies that may be associated with coarctation of the aorta, such as other forms of left heart obstruction. Fetal echocardiography is indicated during pregnancy in every woman who has previously borne a child with congenital heart disease (CHD), especially left heart obstructive lesions.
  • Postnatally, early detection of coarctation of the aorta is important to avoid prolonged hypertension or other complications. Careful measurement of BPs and evaluation of lower extremity pulses in newborns and at subsequent healthy child examinations is imperative.

Complications

Coarctation of the aorta  is a lifelong disease with complications that may not be evident until many years following an initial and apparently successful repair.

  • Recurrent coarctation
    • Recurrence of coarctation is associated with patient size, age at surgery, and associated transverse arch or isthmic hypoplasia. Ductal tissue in the wall of the aorta may involute and contribute to recurrence, as might scarring at the repair site. Some surgeons believe that the use of interrupted sutures in the anterior portion of the anastomotic suture line improves aortic growth and reduces the risk of recurrence. Sometimes, the surgical repair site is unobstructed, yet obstruction develops at the transverse arch or isthmus because of the failure of these areas to grow proportionally to the rest of the arch. Such obstruction may not be detected for many years after initial repair.
    • Some patients who have undergone initial repair with left subclavian flap aortoplasty may have a tortuosity at the repair site that does not result in obstruction until rapid growth in adolescence.
  • Aortic aneurysm
    • Aneurysm of the aorta can occur in unrepaired coarctation of the aorta and has been described in patients with Turner syndrome and coarctation of the aorta. In addition, endocarditis can result in aortic arch aneurysm (mycotic aneurysm), usually distal to the site of obstruction.
    • Patch repair of coarctation of the aorta results in an increased incidence of aortic aneurysm (usually opposite the site of the patch), particularly if the shelf of coarcted tissue is excised. Patients with aortic aneurysm can be entirely asymptomatic. Hoarseness that results from stretching of the recurrent laryngeal nerve associated with aortic aneurysm has been described. Chest radiographs are unlikely to reveal the aortic aneurysm, but MRI is more useful in delineating the size and extent of aneurysms.
  • Hypertension: Hypertension may persist, even after successful repair of coarctation of the aorta, and usually relates to the duration and severity of preoperative hypertension. This is probably related to alterations in the renin-angiotensin system and baroreceptors. As with other forms of uncontrolled hypertension, patients may be at risk for premature atherosclerosis, ventricular dysfunction, and rupture of cerebral aneurysms.
  • Cerebral aneurysms: Berry aneurysms of the circle of Willis or other vessels are believed to occur in as many as 10% of patients with coarctation of the aorta and may be multiple. Aneurysm size tends to increase with age, as does the risk of rupture. Uncontrolled hypertension promotes the growth of the aneurysms and increases risk of rupture. Most patients are asymptomatic until rupture occurs, although some aneurysms may leak prior to rupture, resulting in warning symptoms of headache, photophobia, weakness, or other symptoms. Rupture of a cerebral aneurysm is associated with high mortality rates and should prompt repair of both the aneurysm and coarctation.
  • Paralysis
    • Although rare, paraplegia can occur from spinal cord ischemia, resulting from a compromised blood supply to the anterior spinal artery.40,41 Risk of paralysis is increased with reduced arterial collateral vessels, prolonged aortic cross-clamping time, and intraoperative sacrifice of intercostal arteries, as well as other factors.
    • Paralysis is uncommon in the presence of a well-developed arterial collateral supply, emphasizing the importance of assessing collateral arterial flow prior to surgical intervention. Methods to prevent cord ischemia include hypothermia, use of cardiopulmonary bypass, or insertion of a bypass graft (Gott shunt) with partial aortic clamping.
  • Cardiomyopathy is usually present in infants with critical coarctation, especially if additional levels of left heart obstruction, such as aortic stenosis or subaortic stenosis, are present. Some patients may have changes of endocardial fibroelastosis that result in chronic dilated cardiomyopathy, requiring medical management or, in rare cases, cardiac transplantation. Hypertrophic cardiomyopathic changes also may occur, predisposing the patient to subendocardial ischemia, arrhythmias, or congestive heart failure (CHF) related to diastolic dysfunction.
  • Chylothorax: Extensive dissection at surgery may result in disruption of the thoracic duct, leading to chylothorax. Chylothorax is recognized when feedings are instituted postoperatively. Persistent chylous pleural effusions may necessitate long-term chest tube drainage. Some patients respond to dietary therapy with medium-chain triglyceride diet, fat restriction, and/or total parental nutrition. Patients with refractory chylothorax may require pleurodesis or thoracic duct ligation.
  • Postcoarctectomy syndrome:43,44,45 Restoring pulsatile blood flow to the mesenteric arteries may result in mesenteric arteritis, in which the arteries become distended and may rupture. Reflex arteriolar vasoconstriction occurs as part of autoregulation of blood flow and can result in ischemia. Clinical manifestations may range from mild abdominal discomfort to an acute abdomen with severe abdominal distention, vomiting, ileus, and progression to intestinal wall hemorrhage or perforation. This syndrome may be related to early return to feeding after coarctation repair. Thus, feedings are usually delayed for 48 hours after surgery, and nasogastric tube decompression is continued until feedings are begun slowly and advanced as tolerated. Patients with severe postcoarctectomy syndrome may require exploratory laparotomy for treatment of bowel necrosis or perforation. Careful monitoring and good control of BP in the postoperative period may reduce the risk of postcoarctectomy syndrome.
  • Valvar aortic stenosis, membranous subaortic stenosis, and mitral valve stenosis: These may develop during the follow-up period; if these are significant, transcatheter or surgical relief of the obstruction may become necessary.

Prognosis

  • CoA is a lifelong disease with a guarded prognosis. Relief of obstruction, control of hypertension, follow-up monitoring for recurrent obstruction, and follow-up care of associated anomalies are imperative.
  • Continue subacute bacterial endocarditis prophylaxis indefinitely, even in the absence of associated abnormalities.
  • Patients without residual obstruction who are normotensive both at rest and with exercise should lead normally active lives without restriction. They should be able to obtain health and life insurance.
  • Patients with persistent hypertension, untreated residual obstruction, or other complications have a variable prognosis related to the severity of these problems.
  • Data regarding long-term follow-up are limited. Available studies indicate that significant mortality rates are found at long-term follow-up after surgical correction of aortic coarctation (Rao, Secondary Forms of Hypertension, 1995). The survival curve, while not approaching that in the healthy population, is significantly improved compared with Campbell's natural history data.150 Death appears to be secondary to recoarctation repair, aneurysms at site of coarctation repair or at a remote site, congestive heart failure, bacterial endocarditis, and hypertension. Attempts to define factors that affect long-term survival have been made; age at operation and degree and duration of hypertension prior to surgery appear to affect the long-term survival.

Patient Education

  • Educate patients with coarctation of the aorta and their families regarding the need for lifelong follow-up care to detect recurrent coarctation or late complications.
  • Educate patients regarding a healthy lifestyle by instructing them to avoid smoking and obesity, get plenty of aerobic exercise, control BP appropriately, and reduce other factors that influence cardiovascular disease.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose coarctation of the aorta (CoA) remains a significant medicolegal risk. Hypertensive patients may be treated well into adolescence or adulthood without consideration of the diagnosis of coarctation by physicians. When these patients exhibit complications of coarctation, such as a ruptured cerebral aneurysm, medicolegal repercussions may ensue.
  • Patients may refuse the administration of blood products yet need repair. This may be particularly true of infants whose parents refuse the use of blood products. In these patients, consider balloon dilation of a native coarctation to achieve temporary or even permanent relief of obstruction.
  • Physicians may reduce the risk of paralysis by careful attention to documenting the extent of arterial collateral flow prior to surgical repair in patients with native or recurrent coarctation. Multiple precautionary measures, including Gott shunt, may be used if the status of collateral vessels is uncertain.
  • Cardiac catheterization, if necessary to document the extent of collateral vessels, allows intraoperative measures to protect the spinal cord and to avoid paralysis.

Special Concerns

  • After successful repair of coarctation of the aorta, pregnancy should be well tolerated.151 Prenatal care should include careful monitoring of blood pressure (BP), since hypervolemia in pregnancy may contribute to worsening hypertension, especially in patients with some degree of residual obstruction or preexisting hypertension. The presence of other associated lesions, such as aortic valve disease, may further complicate obstetrical management. Anecdotal cases document aortic rupture during pregnancy.
 


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References
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Further Reading

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Keywords

coarctation of the aorta, CoA, left ventricular obstruction, LV outflow tract obstruction, aortic arch obstruction, interrupted aortic arch, left ventricular hypertrophy, left ventricular afterload, aortic obstruction, bicuspid aortic valve, ventricular septal defect, VSD, left heart obstruction, aortic stenosis, heart defects, congenital heart disease, congestive heart failure, CHF, hypertension, perinatal circulatory insufficiency, Takayasu arteritis, transposition of the great arteries, Taussig-Bing anomaly, double-inlet left ventricle, tricuspid atresia, hypoplastic left heart syndrome, right ventricular outflow tract obstructions, tetralogy of Fallot, pulmonary atresia with intact ventricular septum, cerebral aneurysm, treatment, diagnosis

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Contributor Information and Disclosures

Author

P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Julian M Stewart, MD, PhD, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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