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Ann Thorac Surg 2005;80:1098-1100
© 2005 The Society of Thoracic Surgeons

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Case report

Symptoms Plus Family History Trump Size in Thoracic Aortic Aneurysm

Section of Cardiothoracic Surgery and Cardiovascular Anesthesia, Yale University School of Medicine, New Haven Department of Cardiology, Hartford Hospital, Hartford, Connecticut

Accepted for publication February 18, 2004.

^_* Address reprint requests to Dr Elefteriades, Section of Cardiothoracic Surgery, Yale University School of Medicine, New Haven, CT06510; (Email: john.elefteriades{at}yale.edu).

	Abstract

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A 43-year-old woman presented with chest pain of unclear cause. The patient’s mother and brother had suffered aortic dissection. Echocardiography had shown mild dilatation of the ascending aorta at 4.0 cm. Echocardiogram and magnetic resonance imaging were negative for dissection. The patient was taken to the operating room on the basis of her painful symptoms and her family history. Unexpectedly, a localized dissection was found in the ascending aorta (see Fig 2), which was too small for imaging detection. Without preemptive surgery, full-fledged dissection would have likely occurred with attendant short and long-term consequences. This case emphasizes that size criteria pertain to asymptomatic patients, and symptomatic patients with aneurysm require resection regardless of size.

View larger version (116K): [in this window] [in a new window]   Fig 2. Gross appearance of radiographically undetected aortic dissection. (Arrow shows localized dissection.)

	Introduction

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A 43-year-old woman (5 feet 5 inches tall, 116 lbs), who is a medical professor, presented for evaluation of a small thoracic aortic aneurysm.

The patient’s brother suffered aortic dissection at the age of 34, and the patient’s mother also suffered dissection at the age of 56. Both survived urgent surgical intervention at our institution. A maternal uncle died of a cerebral aneurysm.

The patient had been evaluated several years previously for atypical chest pain, occurring several times a month, and confined to a "quarter-sized" area under the breastbone. One month before our evaluation, the patient experienced 12 hours of feeling chest heaviness and a sense of "impending doom." This resolved spontaneously, and she did not seek treatment. One week before our evaluation, the patient had another episode, this time with pleuritic pain felt in the interscapular region and radiating around the left side to the front of her chest. She sought no evaluation at that time.

Her past history was significant for asthmatic bronchitis with her last steroid course 5 months previously, as well as mitral valve prolapse, positive beta II glycoprotein, and benign breast mass. She has suffered four miscarriages and has given birth to 2 healthy children who are now teenagers.

Physical examination was normal with the exception of I-II/VI systolic and diastolic murmurs heard maximally to the right of the upper sternal border. Workup included electrocardiogram (normal), Holter monitor (normal), and thallium stress test (normal). Echocardiography showed mild dilatation of the ascending aorta at 4.0 cm maximal diameter with no evidence whatsoever of intimal flap or aortic dissection. There was mild aortic insufficiency. The aortic contour showed mild effacement of the aortic sinuses. Echocardiography a year previously had shown a maximal ascending aortic dimension of 3.3 cm. A current magnetic resonance imaging was read as normal, "without evidence of aneurysm or dissection" (Fig 1). Cardiac catheterization showed normal coronary arteries with a right dominant configuration, no aortic insufficiency, normal left ventricular function, and mild effacement of the aortic root. These studies had been accrued electively and incrementally over the months that she had been evaluated.

View larger version (103K): [in this window] [in a new window]   Fig 1. (A) Echocardiogram showing moderately dilated ascending aorta, without dissection (left: long-axis view; right: short-axis view). (B) Magnetic resonance imaging scan showing the same (left: axial cut; right: 3D reconstructed view). (C) Aortogram demonstrating mild effacement of the aortic sinuses.

	Comment

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This patient was operated on despite her small aortic size (4.0 cm) and the lack of evidence of an aortic dissection on echocardiography, magnetic resonance imaging, or aortography. The decision for surgery was made on the basis of two distinct prior episodes of chest symptoms, both of which had resolved spontaneously without medical evaluation or treatment, as well as the strong family history. Yet, a definite preexisting aortic dissection was found, which appeared poised to blossom into a full, propagating type A process, with attendant short and long-term sequelae.

Experience is accumulating with aortic valve sparing procedures for ascending aortic aneurysms, and such a procedure could have been considered as an alternative to the traditional composite graft replacement utilized in this case. Also, it is possible that a limited dissection, such as the one in Figure 2, could go on to heal spontaneously without rupture or progression to propagating aortic dissection. Our impression in the operating room was that the lesion was unstable, with continued exposure of the dissection plane to the intraaortic blood pressure. Also it appears likely that the patient would be subject to additional dissection events in the future without extirpation of this aorta bearing, presumably, an inherent connective tissue abnormality. Another question that can be raised is whether intravascular ultrasound could visualize such a lesion preoperatively; this is certainly possible, but this technology for aortic application is not clinically, widely available.

It can be questioned whether the symptoms in this patient originated from the aortic enlargement, from the aortic dissection, or from some other cause. We believe it is likely that the symptoms were from a bona fide aortic origin. We have seen many other patients with pain and a small aneurysm in whom some findings (edema, adhesions, localized dissection, or intramural hemorrhage) validated aortic origin of symptoms.

This case raises important general issues regarding difficult aortic diagnostic cases. Should a patient with pain always be operated on? How can a physician be sure that the pain is of aortic origin? The posture at our institution is to presume aortic cause unless another specific cause can be identified. As elective aortic surgery becomes safer and safer, such a posture becomes increasingly justifiable. What if external exploration of the aorta is negative? Does one open the aorta on cardiopulmonary bypass? Does one explore the aortic arch under deep hypothermic arrest? Our posture would be to resect the moderately dilated aorta with open distal anastomosis, permitting full assessment of the aortic internal lumen.

This case underscores the importance of symptoms in the decision for or against surgery. It is vital to recognize that the size criteria developed at our institution and others [1–3] (ie, elective extirpation of the aneurysmal ascending aorta at about 5.5 cm diameter) apply to asymptomatic aneurysms. Symptomatic patients require surgery regardless of aortic size. A family history of malignant aortic events (as in this case) should further increase the surgeon’s resolve to intervene in symptomatic patients with only moderate aortic enlargement. Otherwise lives have been and will continue to be lost.

	References

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1. Coady MA, Rizzo JA, Hammond GL, et al. What is the appropriate size criterion for resection of thoracic aortic aneurysms? J Thorac Cardiovasc Surg 1997;113:476-491.[Abstract/Free Full Text]
2. Davies R, Goldstein L, Coady M, et al. Yearly rupture/dissection rates for thoracic aortic aneurysms. Simple prediction based on size Ann Thorac Surg 2002;73(1):17-27.[Abstract/Free Full Text]
3. Griepp RB, Ergin MA, Galla JD, et al. Natural history of descending thoracic and thoracoabdominal aneurysms Ann Thorac Surg 1999;67:1927-1930.[Abstract/Free Full Text]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): John A. Elefteriades Umer Darr Peter Barrett Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Elefteriades, J. A. Articles by Barrett, P. Search for Related Content PubMed PubMed Citation Articles by Elefteriades, J. A. Articles by Barrett, P. Related Collections Great vessels