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Medical management of aortic coarctation is feasible & durable in selected patients: a Case Report & Literature Review.

Introduction

Diagnosis of aortic coarctation (AC) in the elderly is extremely rare as more than 90% of the patients die before age of 50 if the disease is left untreated. (1) This high mortality can be attributed to stroke, aortic dissection, intracranial bleeding or severe aortic regurgitation. AC is considere one of the secondary causes of hypertension. Currently surgical or endovascular repair is the standard of care. In this report we present and review 11 cases of prolonged survival of AC in the elderly mostly from non-English literature.

Case Report

A 84 year old patient who was first seen in our out-patient clinic in 2000. He presented with a history of longstanding but controlled hypertension for more than 30 years. The patient was turned down from enrollment in the army in the early 1950's during the Korean War because he had a heart murmur on physical exam. The patient was subsequently diagnosed with aortic coarctation and was deemed unfit for surgical intervention. He was advised by his doctor at the time not to undergo surgery because of good compensatory flow.

The patient has history of sick sinus syndrome with a permanent pacemaker implantation in 1995. He did not have any history of smoking or alcohol use. He used to work as a gunsmith and is living a retired life now. He walks 1 mile three times a week. His current antihypertensive medications are Atenolol 100mg twice daily, Amlodipine 10mg once daily, Clonidine 0.1mg twice daily, Triamterene/ HCTZ 37.5/25mg half tablet daily and Doxazosin 4mg daily. Work-up for other causes of secondary hypertension was essentially negative.

Upon physical examination, his blood pressure was 180/90 in the upper arm and 160/70 in lower extremities. Grade 1-3 ejection systolic murmur was audible.

Femoral pulses were slightly delayed and pedal pulses were brisk. The rest of the physical exam was unremarkable. EKG showed left atrial hypertrophy with poor R wave progression. Echocardiogram showed an ejection fraction of 5560% and mild aortic regurgitation. A CT angiogram was performed in 2006 which showed complete obstruction of proximal aspects of the descending aorta with well established collaterals (fig. 1). The patient has been followed in our clinic since 2000. The patient's blood pressure remains reasonably well controlled.

Discussion

Coarctation of aorta is a common malformation among newborns accounting for 6 to 8% of congenital heart defects. AC occurs 2 to 5 times more frequently in males. The presentation of AC in the elderly population is rare and approximately 90% of those patients will die by the age of 50 if left untreated. (1) AC is associated with a bicuspid aortic valve in 50% of cases. Coarctation of aorta has been described up to the age of 92 years. (2)

Since an early description by Quain in 1848, (3) AC is considered one of the correctable causes of hypertension in children. Past autopsy studies suggest that the mortality rate in patients in whom coarctation of the aorta is not surgically repaired is 90% by age 50 years, with a mean age of 35 years. (4) The acquired form of coarctation of aorta is commonly associated with inflammatory conditions like Takayasu arteritis (5) and severe atherosclerosis. (6) In Takayasu arteritis mid thoracic or abdominal aorta is commonly involved.

In adults if previously undiagnosed it almost invariably presents as hypertension. Hypertension in adults results in left ventricular hypertrophy and cerebral hypertension. Elevated cerebral pressure can present as headache, cerebrovascular accidents and intracranial bleeds. Patients with coarctation have increased frequency of intracranial aneurysms (7) which can lead to intracranial bleeds and subarachnoid hemorrhage (8) usually presenting between the age of 10 to 30 years. Reduced circulation distal to the coarctation can present as abdominal pain and lower extremity claudication.

Various modalities can be used to diagnose coarctation of aorta but given the accuracy of non invasive methods such as MR/CT angiography they are frequently employed. MR/CT angiography clearly defines the location and severity of the lesion and also helps establish the collaterals. (9) As recommended in the 2008 ACC/AHA adult congenital heart disease guidelines every adult patient with coarctation (repaired or not) should have at least one cardiovascular MRI or CT scan for complete evaluation of the thoracic aorta and intracranial vessels. (10)

Hypertension should be controlled with beta blockers, ACE inhibitors or Angiotensin receptor blockers as first-line medications. The choice of beta blockers or vasodilators may be influenced in part by the aortic root size, the presence of AR, or both. (11,12)

Surgical approach is widely used and varies from open bypass grafting of the occluded aortic segment with interposition graft to minimally invasive catheter based therapy with covered stent. Surgery or endovascular repair is the standard of care for AC. However continued medical management may be an acceptable option for patients with high mortality risk presenting later in life with AC. Complications of interventional repair increases with age as shown by the first long-term follow-up study which reported a 12% death rate in a cohort of 194 patients after a mean duration of 8.8 years. The mean age of death was 35.1 years, and mortality was especially high for those undergoing surgery after the age of 25 years. (13)

Conclusion

While repair of coarctation of aorta by surgery or catheter intervention is the current standard of care, it is worth noting prolonged survival is possible in patients with severe aortic coarctation. Appreciation of this fact may influence management decisions in older patients with coarctation especially if severe comorbidities are present. Our case report highlights the feasibility of medical treatment to achieve proper control of hypertension in cases of complete aortic coarctation. This option may be the only management in a selected group of patients when surgical intervention cannot be offered.

References

(1.) Aboaf AP, Teitelbaum I. Coarctation of the Aorta in the Elderly: Case Report and Review of the Literature. Am J Geriatr Cardiol 1994; 3(1):22-25.

(2.) Abbott, M. E.: Coarctation of the adult type. Am. Heart J. 3: 574, 1928.

(3.) Koksal C, Demirci S, Koksal GM, Zengin M. An infrarenal abdominal aortic coarctation. Surg Radiol Anat 2005; 27: 71-3.

(4.) Campbell M. Natural history of coarctation of the aorta. Br Heart J. Sep 1970; 32(5):633-40

(5.) Pagni S, Denatale RW, Boltax RS. Takayasu's arteritis: the middle aortic syndrome. Am Surg 1996; 62:409.

(6.) Sheikhzadeh A, Giannitsis E, Gehl HB, et al. Acquired thromboatheromatous coarctation of the aorta: acquired coarctation of the aorta. Int J Cardiol 1999; 69:87.

(7.) Perloff JK. Clinical Recognition of Congenital Heart Disease. WB Saunders, Philadelphia 1994.

(8.) Hodes HL, Steinfeld L, Blumenthal S. Congenital cerebral aneurysms and coarctation of the aorta. Arch Pediatr 1959; 76:28.

(9.) Teien DE, Wendel H, Bjornebrink J, Ekelund L. Evaluation of anatomical obstruction by Doppler echocardiography and magnetic resonance imaging in patients with coarctation of the aorta. Br Heart J 1993; 69:352.

(10.) Tobian L Jr. A viewpoint concerning the enigma of hypertension. Am J Med 1972; 52:595.

(11.) Warnes CA, Williams RG, Bashore TM et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714-833

(12.) Baumgartner H, Bonhoeffer P, De Groot NM et al, Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC). Guidelines for the management of grown-up congenital heart disease. The Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC) endorsed by the European Pediatric Cardiology (AEPC). Eur Heart J. 2010 Dec; 31(23):2915-57

(13.) Maron BJ, O'Neal-Humphries J, Rowe RD, Mellits ED. Prognosis of surgically corrected coarctation of the aorta: a 20 year postoperative appraisal. Circulation 1973; 47:119-26

(14.) Raynaud A: Observation d'une obliteration presque complete de l'aorte, suivie de quelques reflexions, et precedee de l'indication des faits analogues consignes dans les auteurs. J Hebd Med (Paris) 1828; 1:161-176.

(15.) Stone JR: Coarctation of the aorta with poststenotic aneurysm in a 71-year-old man. JAMA 1965; 194:1017-1018.

(16.) Miro O, Jimenez S, Gonzalez J, De Caralt TM, Ordi J. Highly effective compensatory mechanisms in a 76-year-old man with a coarctation of the aorta. Cardiology 1999; 92:284-6

(17.) O'Byrne E, Mather J, Hargreaves MR: Coarctation of the aorta: An unusual cause of hypertension in a 73-year-old woman. Int J Clin Pract 1997; 51:466-467.

(18.) Convens C, Vermeersch P, Paelinck B, Van den Heuvel P, Van den Branden F: Aortic coarctation: A rare and unexpected cause of secondary arterial hypertension in the elderly. Cathet Cardiovasc Diagn 1996; 39:71-74.

(19.) Bartlett LC: Coarctation of the aorta in an elderly man. Can Med Assoc J 1983; 129:529.

(20.) Haldane JH: Coarctation of aorta in an elderly man. Can Med Assoc J 1983; 128:1298-1299.

(21.) Liberthson RR, Pennington DG, Jacobs ML, Daggett WM: Coarctation of the aorta: Review of 234 patients and clarification of management problems. Am J Cardiology 1979; 43:835 840.

(22.) Reifenstein GH, Levine SA, Gross RE: Coarctation of the aorta. A review of 104 autopsied cases of the 'adult type', 2 years of age or older. Am Heart J 1947; 33:146-168

(23.) Cevik S, Izgi C, Cevik C. Asymptomatic severe aortic coarctation in an 80-year-old man. Tex Heart Inst J 2004; 31: 429-31

(24.) Kountouris E, Potsis T, Nikas D, Siogas K A severe coarctation of aorta in a 72-year-old female: a case report. Cases J. 2009 Jun 15; 2:6308

Asif N. Khan, MD

Internal Medicine Resident, Charleston Area

Medical Center, Charleston, WV

William Carter, MD

Professor of Medicine, Robert C. Byrd Health

Sciences Center, West Virginia University

Albeir Y. Mousa, MD

Assistant Professor, Dept. of Surgery, Robert C.

Byrd Health Sciences Center, West Virginia

University

Corresponding Author: Ibeir Y. Mousa, MD, Robert C. Byrd Health Sciences Center, West Virginia University, 3110 MacCorkle Ave., SE, Charleston, WV 25304. Email: amousa@hsc.wvu.edu

Table 1. Current literature review to outline recent
and old case reports medical and surgical treatment
of AC in patients older than 70 years.

Study         Age   Medications           Outcome

Raynaud       92    N/A                   Death from
(14),                                     complicated
1828                                      sacral
                                          decubitus
                                          ulcer,
                                          AC diagnosed
                                          on autopsy
Stone         71    N/A                   Death from
(15)                                      bronchopneumonia
,1965
Miro'         76    Captopril and         Death from
et al.              Doxazosin             nosocomial
(16),                                     pneumonia
 1999                                     shortly
                                          after diagnosis
                                          of CA
O'Byrne       73    Triamterene           Died 6
et al.              , Doxazosin,          months
(17),               Enalapril.            after
1997                Furosemide            diagnosis
                    and Amlodipine
Convens       72    Hydralazine,          Was alive
et al.              Furosemide            20 months
(18),                                     after
1996                                      diagnosis

Barlet        74    Chlorthalidone        Alive for
(19)                , Hydralazine         24 years
, 1983                                    after
                                          diagnosis
                                          in 1959
Haldane       74    Hydrochlorothiazide   Alive for 6
(20)                , Methyldopa          years after
, 1983                                    diagnosis

Liberthson    72    N/A                   Died
et al.                                    during
(21), 1979                                surgical
                                          repair
Reifenstein   76    N/A                   Incidental
et al.                                    death
(22), 1947
Cevik S       80    Beta Blockers,        Was alive
2004,               Diuretic and          one year
(23)                ACE inhibitors        after the
                                          surgical
                                          repair
E             72    Beta-blocker,         Refused
Kountouris          Diuretic, Calcium     surgery,
2009, (24)          channel blocker,      Alive at
                    Angiotensin           1 year
                    receptor blocker      follow up

Study         Degree of
              coarctation

Raynaud       N/A
(14),
1828

Stone         Severe
(15)          coarctation
,1965         on autopsy
Miro'         Complete
et al.        interruption
(16),         of aortic
 1999         lumen on
              autopsy

O'Byrne       N/A
et al.
(17),
1997

Convens       Severe
et al.        coarctation
(18),         distal to
1996          the left
              subclavian
              on coronary
              angiogram
Barlet        N/A
(19)
, 1983

Haldane       Complete
(20)          obstruction
, 1983        on Aortic
              angiography
Liberthson    N/A
et al.
(21), 1979

Reifenstein   N/A
et al.
(22), 1947
Cevik S       Severe
2004,         Coarctation
(23)          on MR
              angiogram

E             Severe
Kountouris    Coarctation
2009, (24)    on CT
              angiogram
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Title Annotation:Case Report & Literature Review
Author:Khan, Asif N.; Carter, William; Mousa, Albeir Y.
Publication:West Virginia Medical Journal
Article Type:Clinical report
Date:Mar 1, 2015
Words:1968
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