Asymptomatic Severe Aortic Coarctation in an 80-Year-Old Man

Abstract

Survival to advanced age is exceptional in patients with unrepaired aortic coarctation. We report the case of a hypertensive 80-year-old man with coarctation of the aorta who was otherwise asymptomatic. Coarctation was suspected when a femoral–radial pulse delay was noted during his routine physical examination. A 25-mmHg systolic blood pressure gradient between the upper and lower extremities was detected. Subsequent magnetic resonance angiography, aortography, and coronary angiography revealed severe coarctation of the aorta, well-developed collateral vessels, and severe coronary artery disease that included the left main artery. Coronary artery bypass grafting was performed, and the coarctation was managed conservatively with antihypertensive medication. At the 1-year follow-up visit, the patient was still asymptomatic and maintained a normal blood pressure with medication.

Coarctation of the aorta is typically a disease of childhood and early adulthood, and there is a reduced life expectancy in patients who have not undergone correction. Survival to older age is rare, due to severe cardiovascular complications.¹ Because there are only a few cases of elderly patients with uncorrected aortic coarctation, management strategies in these patients are controversial. We describe the case of a man first diagnosed with coarctation of aorta at an advanced age.

Case Report

In January 2003, an 80-year-old man was referred to our clinic for evaluation and treatment of recently diagnosed hypertension. He was otherwise asymptomatic. On physical examination, the blood pressure was 170/95 mmHg in both arms. A grade 2/6 systolic murmur was heard in the vicinity of the aorta. Keith-Wagener-Barker stage II hypertensive changes were found on funduscopic examination. The femoral pulses were palpable bilaterally; however, a radial–femoral pulse delay was noted. Lower extremity blood pressure was 145/90 mmHg; hence there was a systolic pressure gradient of 25 mmHg between upper and lower extremities. The patient denied symptoms of claudication and weakness of his legs. Results of routine blood chemistry and urine analysis were normal. Electrocardiography revealed left ventricular hypertrophy. Concentric left ventricular hypertrophy, a normal tricuspid aortic valve, normal left ventricular systolic function, and a turbulent jet at the distal aortic arch were observed on echocardiographic examination. Although it is extremely rare at this age, aortic coarctation was suspected and thoracic magnetic resonance (MR) angiography was performed. The MR angiogram showed severe coarctation of aorta just below the origin of the left subclavian artery, together with post-stenotic dilatation (Fig. 1). Compensatory dilatation of the intercostal arteries was noted. Aortography (Fig. 2A) and coronary angiography (Fig. 2B) were performed via the brachial route, and severe triple-vessel disease with a left main stenosis was detected. Cranial MR did not detect any intracerebral aneurysm.

Fig. 1 Thoracic magnetic resonance angiography shows severe aortic coarctation (arrow) and collateral circulation (arrowheads) from the left subclavian artery to the descending aorta.

Fig. 2 Aortography (A) and left coronary angiography (B) of the patient: severe left main disease is revealed by the absence of contrast reflux (arrow).

The patient underwent a successful coronary artery bypass operation (CABG) with saphenous vein grafts, and we planned to correct the coarctation at a 2nd session. Since he was asymptomatic and his hypertension was well controlled with a combination of antihypertensive agents (β-blocker, diuretic, and angiotensin-converting enzyme inhibitor), the patient refused the 2nd operation. At his 1-year follow-up visit, he was doing well, with good effort capacity and blood pressure of 145/85 mmHg. He was informed of the substantial risk of infective endocarditis and of the need for prophylaxis.

Discussion

Aortic coarctation is a congenital malformation of the aorta that should be diagnosed and corrected early in life. The natural history of unrepaired coarctation of the aorta includes the development of systemic hypertension and subsequent morbidity and death from cardiovascular disease. If the condition is left uncorrected, most patients die by the 5th decade of life, and very few survive to older age.¹ The age at correction is the most important factor for the relief of hypertension and long-term survival.² Persistent hypertension is a substantial problem after surgical repair of aortic coarctation and is more frequent in patients who undergo surgery at a more advanced age. Bouchart and colleagues³ reported long-term follow-up results in 35 patients who underwent coarctation repair at an age older than 20 years. In their series, all 5 of the patients who were operated on at 40 years or older remained hypertensive and needed antihypertensive medication at late follow-up.

There are few reports of patients first diagnosed with uncorrected coarctation at very late age (>60–70 years), and there is no consensus on how to manage them. Two patients—one age 72⁴ at the time of the report and the other age 76⁵—were followed up without repair. The former had undergone a CABG operation and was asymptomatic at 2-year follow-up, and the latter died of pneumonia unrelated to the coarctation. In contrast, some surgeons have performed successful correction in these older patients.^6–8 Aris and coworkers⁷ and Bauer and co-authors⁸ reported good surgical outcomes for coarctation repair in 8 and 15 patients, respectively, who were older than 50 years; however, only 2 and 3 patients, respectively, were older than 60 years. Although hypertension was controlled more effectively after the repair, most of the patients still needed antihypertensive medication at late follow-up. These studies demonstrated that symptomatic improvement occurred, and hypertension was well controlled after correction in these older patient groups. However, whether patients older than 60 to 70 years gain any survival benefit from coarctation repair is still largely unknown.

Survival of a patient to 80 years of age is extremely exceptional in cases of uncorrected coarctation. Because coronary artery disease is a significant cause of death in these patients and coronary angiography revealed that our patient had left main coronary artery disease, we performed CABG. We had planned to correct the coarctation at a 2nd session; however, the well-controlled hypertension and relatively low transaortic gradient with well-developed collateral vessels, together with the patient's unwillingness to undergo further treatment, led us to be conservative. It is doubtful whether the correction would have prolonged the patient's survival or improved his quality of life.

Although single-stage correction of coarctation in combination with CABG has been reported,^7,9 we think that this would have carried far too much perioperative risk in our very elderly patient. One of the 8 patients of Aris and associates⁷ who underwent single-stage combined coarctation and CABG surgery developed postperfusion lung edema that necessitated mechanical ventilation. Similarly, Pethig's group¹⁰ reported the cases of 2 patients in whom weaning from extracorporeal circulation required resuscitative measures, after single-stage combined coarctation repair and aortic valve replacement. They proposed that changes in the hemodynamics of the thoracic vascular bed resulted in coronary malperfusion, which led to early postoperative heart failure and life-threatening ventricular arrhythmias in these patients.

Transcatheter balloon dilation with or without stent implantation is now being performed increasingly for treatment of coarctation and is recommended as the therapy of choice in experienced centers.^11,12 Favorable early, medium-term, and long-term results have been reported in adolescent and adult coarctation patients,¹² but experience and evidence of benefit in very old patients is scarce.

In conclusion, patients with aortic coarctation rarely survive to old age for their first diagnosis. Elderly patients with uncorrected coarctation should be examined and treated for possible complications of coarctation, such as hypertension, coronary artery disease, and cerebral aneurysms. Although technically more challenging, anatomic correction can also be considered in elderly patients and can yield good surgical results.^6–8 Transcatheter intervention is also emerging as a safe and effective alternative to surgery in centers experienced with this procedure. However, the beneficial effect of intervention—whether surgical or transcatheter—in terms of diminished mortality in very old patients is still questionable, which makes conservative management with antihypertensive drug therapy an acceptable treatment option in such patients.