Asian Cardiovasc Thorac Ann 2006;14:522-524
© 2006 Asia Publishing EXchange Ltd
Cardiac Transplantation and Aortic Coarctation Repair in Severe Heart Failure
O Christopher Raffel, FRACP,
Arun Abraham, MB ChB,
Peter N Ruygrok, MD,
A Kirsten Finucane, FRACS1,
Alastair D McGeorge, FANZCA2,
Renelle L French, NZCS
Department of Cardiology
1 Department of Cardiothoracic Surgery
2 Department of Anesthesia, Green Lane Hospital, Auckland, New Zealand
For reprint information contact: Peter Ruygrok, MD, Tel: 64 9 307 4949 Extn 23675, Fax: 64 9 9 631 0733, Email: Ruygrok{at}adhb.govt.nz, Green Lane Cardiovascular Services, Level 3, Building 32, Auckland City Hospital, Private Bag 92 024, Auckland, New Zealand.
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ABSTRACT
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A 37-year-old man presented with severe dilated cardiomyopathy secondary to occult aortic coarctation. He was successfully managed with combined orthotopic heart transplantation and aortic coarctation repair.
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INTRODUCTION
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Cardiomyopathy as a consequence of aortic coarctation has been previously reported; however, its incidence is unknown. This combination presents a difficult management problem.
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CASE REPORT
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A 37-year-old man presented to his local hospital with a 1-month history of increasing dyspnea, orthopnea, and nocturnal dyspnea. He had noticed a gradual limitation in exercise tolerance over the previous 6 years. He was known to have a bicuspid aortic valve at the age of 14 years and was treated for bacterial endocarditis at the age of 23 years, after which he had no contact with any medical services. He gave a 10-pack-year smoking history and consumed alcohol in moderation. He had no significant family history and was not on regular medication. On admission, he appeared unwell, tachypneic, and had a tachycardia of 110 beat·min–1. He was afebrile. His blood pressure was 105/60 mm Hg and his peripheral pulses were of low volume. Jugular venous pressure was elevated at 10 cm, but he had no peripheral edema. Auscultation revealed a gallop rhythm with an apical 3rd heart sound, a 2/6 pansystolic murmur at the left sternal edge, and widespread bilateral inspiratory pulmonary crackles. An electrocardiogram showed sinus tachycardia, left ventricular (LV) hypertrophy, and LV strain pattern. Chest radiography demonstrated cardiomegaly with interstitial pulmonary edema. A full blood count, serum electrolytes and creatinine, erythrocyte sedimentation rate, and C-reactive protein were normal. Blood cultures were sterile. Transthoracic echocardiography revealed biventricular dilatation with severe biventricular systolic dysfunction. The LV ejection fraction was estimated as 15%. There was moderate functional mitral and tricuspid regurgitation. The bicuspid aortic valve was competent with no significant stenosis.
A diagnosis of severe dilated cardiomyopathy with decompensated cardiac failure was made, and intravenous diuretic therapy was commenced. There was little improvement in the patient’s condition over the next 48 hr and he was transferred to our institution for further management. On arrival, he was in a state of low output cardiac failure. In the context of his bicuspid aortic valve, the possibility of occult aortic coarctation as the cause of his heart failure was considered. Although his peripheral pulses were generally weak, his femoral pulses were almost impalpable with an upper limb-lower limb differential systolic blood pressure of 52 mm Hg. Chest radiography revealed an absent aortic knuckle with notching of the 3rd to 5th ribs. A repeat transthoracic echocardiogram confirmed the findings of the initial study and showed an abnormally shaped aortic arch with posterior indentation. Continuous-wave Doppler interrogation of the descending aorta indicated forward flow throughout diastole and a peak velocity of 2.8 m·s–1, consistent with aortic coarctation. Investigations of other causes of cardiomyopathy were negative. The patient’s condition improved with intensive medical therapy but he remained significantly limited with New York Heart Association functional class III IV symptoms.
Cardiac catheterization and aortography confirmed severe aortic coarctation (Figure 1
) with a peak-to-peak systolic gradient of 41 mm Hg across the coarctation (Figure 2). The cardiac index was 2.0 L·min–1·m–2 and pulmonary arterial resistance was 3.5 Wood units. Coronary angiography was normal. That same day, a suitable donor heart became available and the patient underwent combined coarctation repair and heart transplantation via a median sternotomy. The heart was severely dilated and there was a tight coarctation with a lumen diameter of 3 mm just beyond the origin of the left subclavian artery. Beyond the coarctation, the descending aorta was aneurysmal. Several large collateral vessels were noted. Using aortic and bicaval cannulation, bypass was commenced, and the heart was cooled to 15°C. Full dissection of the arch, isthmus, and descending aorta was performed. Access to the aneurysmal area was achieved by mobilizing the left pulmonary artery and left bronchus downwards, and improved when the aorta was crossclamped and the heart deflated. During 45 min of circulatory arrest, the coarctation segment and distal calcified aneurysmal portion of the descending aorta were excised and repaired using a 24 mm Hemashield Dacron graft (Meadox Medicals, Inc., Oakland, NY, USA). Once back on bypass and rewarmed, the patient’s heart was resected and the donor heart was inserted in a routine manner with bicaval anastomoses, giving intermittent doses of blood cardioplegia. Bypass time was 3 hr 12 min and donor heart ischemic time was 3 hr 48 min. Histology of the ventricular myocardium from the explanted heart revealed subendocardial myofiber hypertrophy with minimal associated interstitial fibrosis and no inflammation. The patient’s postoperative recovery was rapid and uneventful. Two episodes of moderate rejection were successfully managed with pulses of intravenous methylprednisolone. Twelve months after the operation, he remained well with normal exercise tolerance.
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Figure 1. Aortogram in left anterior oblique projection showing severe coarctation of the descending aorta.
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Figure 2. Ascending and descending aorta phasic pressure tracings showing a significant systolic pressure gradient across the coarctation; as a result of the severe coarctation the normal phasic pressure changes in the descending aorta are significantly blunted.
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DISCUSSION
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Heart failure is common in untreated aortic coarctation, having a bimodal age distribution and occurring most frequently in infants and in adults over the age of 40.1 Heart failure is the most common cause of death in untreated coarctation, with a mortality of 90% before the age of 50 years.1 While dilated cardiomyopathy as a consequence of coarctation in adults has been reported, the frequency of its occurrence is unknown.2–4 Nevertheless, it should be considered in the differential diagnosis of causes of heart failure and cardiomyopathy in adults. The absence of upper-limb hypertension and the generalized low-volume peripheral pulses in our patient probably contributed to the fact that the diagnosis of coarctation was not initially entertained. These findings were likely due to a combination of extensive collateralization and low cardiac output. In a series of 84 patients with coarctation, up to 40% had upper limb normotension.5 The presence of a bicuspid aortic valve has been observed in 25% 85% of patients with aortic coarctation, and should raise the possibility of its presence, as in our patient.6
There are no good data to guide the management of a patient with severe cardiomyopathy secondary to coarctation. While many surgical series of coarctation repair included adult patients with heart failure, no objective data on pre and postoperative LV systolic function were included.1,7 Isolated coarctation repair would have been a high-risk procedure in our patient. Even if he had survived the operation, it is likely that he would have retained significant LV dysfunction and symptomatic heart failure. In a series of 16 adults 
40 years with preoperative heart failure, 6 had persistent heart failure following coarctation repair.1 A 53-year-old man with dilated cardiomyopathy and severe systolic dysfunction secondary to coarctation underwent coarctectomy; his symptoms of heart failure resolved with a modest improvement in LV dimensions, but there was no obvious improvement in LV function 2 months after surgery.2 There is only one other report of simultaneous coarctation repair and heart transplantation in the English language literature: a 59-year-old man with dilated cardiomyopathy, coarctation, and LV ejection fraction < 15% underwent successful orthotopic heart transplantation and ascending aorta-descending aorta bypass with a Hemashield graft.3
Data continue to accumulate on transcatheter intervention in adults with native aortic coarctation, with some authors suggesting that catheter-based therapy should be the treatment of choice in most adults.8 With increasing age at operation being the most important predictor of adverse outcome following surgical repair, percutaneous treatment is an attractive alternative. This would be particularly appealing in a patient with significant comorbidity where a potentially simpler and lower-risk procedure would be advantageous. Transcatheter treatment of coarctation without cardiac transplantation would be an alternative strategy in a patient who has a secondary cardiomyopathy, with the hope that LV function and symptomatic status would improve after relief of the coarctation. This was not undertaken in our patient because of the severity of his heart failure and because the coarctation was felt to be unsuitable for percutaneous treatment. The case of a 56-year-old woman with coarctation and secondary cardiomyopathy who was successfully managed by deploying a Palmaz stent across the stenosis has been described.4 She remained well and her LV systolic function improved from 31% to 41% 1 year after the procedure. Unfortunately, she died at 22 months postoperatively from a sudden cardiac event. No postmortem data were given.
Occult aortic coarctation should be considered in the differential diagnosis of causes of cardiomyopathy in adults. Our experience indicates that in this small group of patients with severe heart failure and aortic coarctation, if percutaneous treatment is unsuitable or fails, they can be successfully managed with simultaneous coarctation repair and orthotopic heart transplantation.
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ACKNOWLEDGMENTS
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Thanks to Shingo Kasahara, MD, and Denise M Reddy, RN, for their assistance in preparing the manuscript.
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REFERENCES
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- Liberthson RR, Pennington DG, Jacobs ML, Daggett WM. Coarctation of the aorta: review of 234 patients and clarification of management problems. Am J Cardiol 1979;43:835–40.[Medline]
- Pauly DF, Morss SE, Tanio JW, Irani K, Cameron DE, Schulman SP, et al. Reduced left ventricular dimension and normalized atrial natriuretic hormone level after repair of aortic coarctation in an adult. Clin Cardiol 1999;22:233–5.[Medline]
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- Glancy DL, Morrow AG, Simon AL, Roberts WC. Juxtaductal aortic coarctation. Analysis of 84 patients studied hemodynamically, angiographically, and morphologically after age 1 year. Am J Cardiol 1983;51:537–51.[Medline]
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- Hornung TS, Benson LN, McLaughlin PR. Interventions for aortic coarctation. Cardiol Rev 2002;10:139–48.[Medline]
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ABSTRACT
INTRODUCTION
