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J R Soc Med. 2004 June; 97(6): 283–284.
PMCID: PMC1079493

Left arm pain 22 years after repair of aortic coarctation

Aqeel Bhutta, MB BSc and Akhtar Nasim, MD FRCS

In children, coarctation of the aorta is often repaired by use of the initial segment of the left subclavian artery as a patch to widen the narrow segment.1 The left arm then relies on development of collateral vessels.

CASE HISTORY

A woman of 22 sought advice about a cold left hand and exercise-related left arm pain. She was a postgraduate student and had found it difficult to type her thesis. There was a history of cardiovascular surgery in early life. At the age of three weeks a localized pre-ductal aortic coarctation had been diagnosed in association with a patent ductus arteriosus and a large ventricular septal defect (VSD). Initially, the aortic coarctation had been repaired by a subclavian flap angioplasty technique (this included ligation of the left vertebral artery), with simultaneous ligation of the ductus arteriosus. 4 years later the persistent VSD had been repaired.

On examination, her left arm was normally developed but the hand felt cold and none of the arm pulses was palpable. With a hand-held doppler the left brachial pressure was 95 mmHg compared with 120 mmHg on the right. An angiogram confirmed absence of the left subclavian artery at its origin (Figure 1). The artery re-formed 3 cm distally and was hypoplastic in appearance (Figure 2). There was no recurrence of the aortic coarctation or VSD (on echocardiography). In view of the severity of symptoms, a left carotid to subclavian arterial bypass (with 6 mm diameter Dacron graft) was performed via a single supraclavicular incision, and she was discharged five days later with a warm hand and palpable arm pulses. An arterial duplex scan at two months confirmed that the bypass was functioning, with equivalent arm pressures.

Figure 1
Angiogram showing absence of left subclavian artery at its origin (big arrow)
Figure 2
Angiogram showing refilling of hypoplastic left subclavian artery approximately 3 cm distal to its origin (arrow)

COMMENT

Coarctation of the aorta accounts for 3-8% of all congenital cardiac abnormalities, and is associated with at least one other cardiac abnormality in 59-92% of cases.1,2 Most infants born with a large VSD and coarctation develop severe congestive cardiac failure in the first month of life, necessitating urgent cardiac surgery.3,4 The repair of aortic coarctation in neonates and infants can be undertaken either by resection and end-to-end anastomosis or by use of the proximal left subclavian artery to patch and widen the coarctation.1,2,4 The latter technique was introduced in 1966 and is associated with better survival and lower rates of recurrence.3 When it is used in neonates and infants, the general view is that adequate collateral circulation develops to prevent any clinically important morbidity, with an adverse event rate of around 0.2%.3,5 However, there are a few reports of impaired left arm blood flow leading to limb shortening, decrease in limb girth and mild symptoms of ischaemia on strenuous exercise.4,5

In our patient, symptoms become troublesome 22 years after repair of her coarctation. We have found only one other case report of such late presentation; in that report the patient had an acute juxta-vertebral thrombosis of the subclavian artery which caused symptoms despite a collateral supply from the intercostal arteries, the thyrocervical trunk and the costocervical system.6 The vertebral artery is an important source of collateral supply in the presence of disease at the origin of the subclavian artery. It is not routine practice to ligate this during subclavian flap angioplasty of aortic coarctation. Sacrifice of this at the initial procedure in our patient may account for the inadequate development of collateral supply. With improved long-term survival and the increasing cohort of patients with subclavian flap angioplasty reaching adulthood, clinicians should be aware of their potential susceptibility to arterial insufficiency.

References

1. Attenhofer Jost CH, Schaff HV, Connolly HM, et al. Spectrum of reoperations after repair of aortic coarctation: importance of an individualised approach because of coexistent cardiovascular disease. Mayo Clin Proc 2002;771: 646-53 [PubMed]
2. Nguyen DM, Tsang J, Tchervenkov CI. Aneurysm after subclavian flap angioplasty repair of the aorta. Ann Thorac Surg 1999;68: 1392-4 [PubMed]
3. Isomatsu Y, Imai Y, Shin'oka T, Aoki M, Sato K. Coarctation of the aorta and ventricular septal defect: should we perform a single-stage repair? J Thorac Cardiovasc Surg 2001;122: 424-4 [PubMed]
4. Allen BS, Halldorsson AO, Barth MJ, Ilbawai MN. Modification of the subclavian patch aortoplasty for repair of aortic coarctation in neonates and infants. Ann Thorac Surg 2000;69: 877-80 [PubMed]
5. Diemont FF, Chemla ES, Julia PL, Sirieix D, Fabiani JN. Upper limb ischemia after subclavian flap aortoplasty: unusual long-term complication. Ann Thorac Surg 2000;69: 1576-8 [PubMed]
6. Wells WJ, Castro LJ. Arm ischemia after subclavian flap angioplasty: repair by carotid-subclavian bypass. Ann Thorac Surg 2000;69: 1574-6 [PubMed]

Articles from Journal of the Royal Society of Medicine are provided here courtesy of Royal Society of Medicine Press