Thorax, 1979, 34, 606-611

Dissection of an aneurysmal ascending aorta inassociation with coarctation of the aortaROBERT A M LAWSON AND ANTHONY FENNFrom the Cardio-Thoracic Unit, Wythenshawe Hospital, Manchester, UK

ABSTRACT A previously fit 21-year-old man presented with severe central chest pain. Clinical,electrocardiographic, and echocardiographic examination confirmed a dissection of ananeurysmal ascending aorta in the presence of previously undiagnosed severe aortic coarctation.Initial aortic dissection had occurred five days before admission. The medical and stagedsurgical management of the case are presented. Surgical survival with such a combination oflesions does not appear to have been previously recorded.

In a classic paper on 200 necropsy cases of aorticcoarctation collected over 137 years Abbott(1927/8) noted that 20% of the deaths were fromrupture of the aorta or, rarely, of the heart itself.In a later series of 104 necropsies similar aorticrupture accounted for 23% of the deaths (Reifen-stein et al, 1947). Isolated cases of ruptured aorticaneurysm in association with aortic coarctationhave been reported since then, with successful sur-gical management of some of those with rupturedmycotic aneurysms distal to the coarctation (Pro-bert, 1956; Cossette et al, 1969; Nikaidoh et al,1973).We have been unable to trace a recorded case

of successful surgical management of rupture ofthe aneurysmal ascending aorta with coexistingsevere coarctation.

Case report

A previously fit 21-year-old truck driver developedsudden severe central chest pain as he lifted a7 kg carton of margarine. He was admitted tohospital where severe pain persisted for 13 hoursdespite sedation.The patient was in good general condition but

had a coarctation of the aorta with barely palpablefemoral pulses, a well-developed chest wall col-lateral circulation, and an arm blood pressure of180/100 mmHg. A chest radiograph was thoughtto show some cardiac enlargement and rib notch-ing consistent with aortic coarctation but to beotherwise normal.

Pain recurred intermittently after 72 hours inthe central chest and also in the left shoulder and

neck. His circulatory state remained stable,although his blood pressure remained at 150-190/70-100 mmHg. After a further episode of pain hewas transferred to a regional cardiothoracic unitfive days after the onset of his symptoms.On admission to the regional unit his general

condition remained good despite the pain. Hisbuild was normal without any stigmata of Marfan'ssyndrome. The previous clinical findings were con-firmed. An easily palpable thrill was noted overthe right carotid artery and a diastolic murmurwas heard down the left sternal margin. Air entryto the chest was good and abdominal examinationwas normal. His haemoglobin was 13-8 g/dl, whitecell count 13-3 X 109/l, serum electrolytes werenormal, and blood urea 115 mmol/l. Echocardi-ography (figs 1 and 2) showed a large pericardialeffusion, anterior mitral leaflet diastolic flutterindicating severe aortic regurgitation, a vigorousleft ventricle (calculated cardiac output of 14-8 1/min), and some enlargement of the aortic root(diameter 42 mm) but no double aortic wall echo.The ECG showed sinus rhythm, axis of -300,left ventricular hypertrophy, and ST changes con-sistent with pericarditis. The chest radiograph(fig 3) confirmed pronounced cardiomegaly, bi-lateral rib notching, and considerable widening ofthe mediastinum.A clinical diagnosis was made of dissection of

the aneurysmal ascending aorta with aortic re-gurgitation and pericardial effusion in associationwith coarctation of the aorta. Angiography wasnot done at this stage as it was considered to betoo hazardous. We elected to resect the coarctationas an initial emergency procedure and to investi-

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Fig 1 Admission echocardiogram showing a large pericardial fluid collection. Left ventricularaction is vigorous.

gate and deal with the ascending aortic dissectionat a later date. Immediately after admission thepatient's systolic blood pressure was reduced to100 mmHg with intravenous sodium nitroprusside.On 30 November 1977 resection of his aortic

coarctation was carried out through a left fourthrib thoracotomy. A tense bluish pericardial effusionwas confirmed at operation and left severely alone.A dissection of the ascending aorta was seen toextend to, but not beyond, the coarctation thatlay in the classical site just distal to the left sub-clavian artery. After resection of the coarctation,aortic continuity was established with a 4 cm long14 mm diameter woven Dacron graft. Artificialventilation and sodium nitroprusside treatmentwere discontinued 12 hours after operation, armblood pressure thereafter being maintained at110-120/60-70 mmHg with propranolol andmethyldopa.Over the next four days the patient had no more

central chest pain, his general condition remainedstable, and the murmur of the aortic regurgitation

remained unchanged. The chest radiograph,however, showed minimal further mediastinalwideningA left heart study on 5 December 1977 showed

a left ventricular pressure of 130/20 mmHg and anascending aortic pressure of 120/65 mmHg. Anaortic root injection of contrast medium confirmedgross dilatation of the ascending aorta whichnarrowed inferiorly to a slightly enlarged aorticring. Superiorly the aorta became relatively normalin size below the innominate artery origin.Moderate to severe aortic regurgitation into a verydilated left ventricle was shown. No definite dis-section could be seen.

Immediately after this investigation an ex-ploration of the ascending aorta and aortic valvewas begun. After cannulation of the right femoralartery for arterial return, the pericardium wasopened to release 400 ml of old blood and fibrin.The ascending aorta measured 8 cm in diameterand narrowed superiorly and inferiorly. A posteriordissection originating 1-5 cm above the left

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Fig 2 A wide aortic root is shown but there is no clear evidence of dissection. Fine diastolic oscillationof anterior mitral valve leaflet indicates aortic regurgitation.

Fig 3 Chest radiograph on admission to regionalcardiothoracic unit. A considerable increase inmediastinal and cardiac size is noted.

coronary orifice had tracked down to, but notaround it. The dissection had extended leftwardsaround the aorta, but no definite exit point intothe pericardium could be seen anteriorly. Su-periorly the dissection affected three-quarters of

the aortic circumference and had extended up theinnominate artery and beyond it around the arch.The aortic valve was tricuspid and the cusps werehealthy, but regurgitation resulted from anteriordislocation of the commissure between the non-and left-coronary cusps by the inferior dissection.The left ventricle was moderately hypertrophiedand considerably dilated.With the aid of cardiopulmonary bypass, whole

body cooling to 280C, and cold hyperkalaemiccardioplegia the ascending aorta was replaced witha 25 mm preclotted woven Dacron graft after re-suspending the dislocated aortic commissure using4/0 Prolene sutures tied over Teflon buttressesoutside the aortic wall. The proximal end of thegraft was sutured to the aorta 0-5 cm above thecoronary orifices.The cardiopulmonary bypass was terminated

without difficulty. Continued suture line bleedingultimately responded to infusion of fresh bloodand Teflon wrapping of both suture lines.After the operation the circulation was sup-

ported by minimal doses of dopamine for 24 hoursafter which artificial ventilation was discontinued.The patient thereafter made an uneventful re-

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covery and was discharged home 16 days after hissecond operation. He was taking digoxin, 0-25 mg,and propranolol, 30 mg, daily.On discharge he had clinical signs of moderate

aortic regurgitation with a 2/6 diastolic murmurdown the left sternal edge and a blood pressure of130/60 mmHg. Echocardiography (fig 4) showed awide aortic root (43 mm diameter) with eccentricaortic cusp closure, slight left ventricular dilatationwith excellent action, and moderately increasedcardiac output at 9 9 1/min. The chest radiographshowed a striking reduction in his heart size (fig 5).Three months after operation he resumed full-

time work and when seen one year later he wasasymptomatic with a blood pressure of 140/75mmHg in both arms. His chest radiograph showeda further reduction in heart size and his ECGshowed improvement on the previously noted signsof left ventricular strain. His diastolic murmur,however, remained unchanged and a supra-aorticcontrast injection showed moderate-to-severeaortic regurgitation (fig 6).

Histology of the coarctation specimen showedconsiderable thickening of both media and intimawith some atheroma. The lumen was reduced to a2 mm slit at the centre of the specimen. Histologyof the ascending aortic aneurysm showed intimalthickening and extensive haemorrhage into the

Fig 5 Chest radiograph on discharge showing apronounced reduction in mediastinal shadowing andsome reduction in the cardiac shadow.

media. Where the media was present there wasnecrosis with loss of nuclear pattern and hyalinisa-tion of the cytoplasm. The adventitia was infil-trated with inflammatory cells, mainly polymorphsbut with some plasma cells and leucocytes. Theappearances were compatible with dissectinganeurysm of the aorta associated with medialnecrosis.

Fig 4 Echocardiogram on discharge shows a wide aortic root with eccentric aortic valve closure. Theleft ventricle is vigorous and fine mitral oscillations are again shown.

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Fig 6 Supra-aortic contrast medium injection one

year after operation showing moderate-to-severe aorticregurgitation.

Discussion

The natural history of untreated aortic coarctationhas been well reviewed by Abbott (1928/29) andReifenstein et al (1947). Of the deaths in theseseries, 75% were from complications of the aorticcoarctation, of which aortic rupture accounted forabout 20%. Rupture, unassociated with infection,usually occurred in the ascending aorta and rarelyin the descending aorta. When, much less often,the rupture was associated with infection, it couldoccur in either the ascending or more often in thedescending aorta. Thus 52 of their 62 ruptured,non-infected aneurysms occurred in the ascendingaorta and 10 in the descending aorta. Death afterascending aortic rupture was almost invariablysudden, only three of 52 patients surviving for morethan three days.Our patient was fortunate enough to survive the

immediate dissection, a five-day hospital stay, anda 10-mile ambulance trip to the regional unitbefore receiving specific and effective hypotensivetreatment. He then survived an emergency resec-

tion of his coarctation under hypotensive controland a further four days in hospital under hypo-tensive control until resection of his ascendingaorta was performed.Management of dissection of the ascending

aorta in the presence of severe aortic coarctationposes problems for both the investigating cardi-

ologist and the surgeon. Dissection of the ascend-ing aorta in these cases classically originatesposteriorly (Abbott, 1928/9), extends as far as thecoarctation (Edwards, 1973), and may affect headand arm vessels, as occurred in our case. Extremelyweak and delayed femoral pulses and gross chestwall collaterals confirmed a severe aortic coarcta-tion and contraindicated any catheter study orarterial cannulation from the groin. Echocardio-graphy confirmed a large pericardial effusion,aortic root dilatation, and anterior mitral cusposcillation secondary to aortic regurgitation butdid not show a definite dissection. We consideredthat the clinical, radiological, electrocardiographic,and echocardiographic findings in our patient weresufficiently definite to make a firm diagnosis ofdissection of the ascending aorta in the presenceof severe aortic coarctation and that angiographicstudies at this stage might prove more hazardousthan helpful.

Pharmacological (Wheat et al, 1965) and ana-tomical reduction in after-load by resection of theaortic coarctation seemed the logical initial man-agement. We accepted the considerable risks ofdelay in resection of the dissected ascending aorta(Daily et al, 1970; Dalen et al, 1974; Strong et al,1974). Controlled hypotension during coarctationresection was mandatory to avoid damage byaortic clamps to the upper aorta surrounded bymediastinal haematoma. From previous experiencewe had learnt to resist the temptation to releasea haemorrhagic pericardial effusion as this canresult in massive bleeding as the aortic tear is de-compressed. The haemorrhagic effusion should bewidely released only with an arterial return can-nula in place so that cardiopulmonary bypass canbe immediately started should the aorta rupture.The second operation was delayed until we

thought that our suture lines after resection of thecoarctation could successfully withstand the hep-arinisation needed for cardiopulmonary bypass.We would have expedited the second stage hadthe patient complained of any central chest painor had there been any difficulty in controlling hisblood pressure.

Aortic valve repair was performed on ourpatient in view of his youth and the presence of atricuspid valve with healthy cusps that appearedto approximate centrally and to be competent afterresuspension of the prolapsed commissure. Tefloncloth insertion into the inferior dissection to pro-vide a fixed annulus was not used (Collins andCohn, 1973). Our patient's moderate-to-severepostoperative aortic regurgitation may be relatedto some dilatation of the annulus or possible dis-

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tortion, or both, caused by the external Teflonwraps used to control suture line bleeding.

Medial necrosis in the ascending aorta adjacentto areas of the aortic rupture was noted in 12 ofthe 13 patients in Abbott's series on whom histo-logical examinations were performed. Interruptionand diminution of the elastica with connectivetissue increase and hyaline and fatty degenerationwere specifically noted. The frequent finding of adilated ascending aorta, especially thin walled atits root and associated with a bicuspid aortic valvein 50% of her cases suggested to Abbott a "pri-mary deficiency in the vascular anlage." Medialnecrosis with hyaline degeneration, basophilic ap-pearance, or cystic change was also noted byReifenstein and co-workers (1947).An association between cystic medial necrosis

and bicuspid aortic valve has also been noted(McKusick et al, 1957) and thought to be similarto that between coarctation and bicuspid aorticvalve (McKusick, 1972a). Although the medialnecrosis was at first thought to be the result of anon-specific aortic response to haemodynamicstress (McKusick et al, 1957), similar histologicalfindings in arteries under less haemodynamic stress-for example, the pulmonary artery, and in theascending aorta of a father and son who both hadbicuspid aortic valves and died after rupture oftheir ascending aortic aneurysm-have subse-quently caused McKusick (1972b) to suggest thatthe aetiology may be congenital.Gross aneurysms of the sinuses of Valsalva have

been recorded as developing within two years ofreplacement of the aortic valve and ascendingaorta in a teenager with Marfan's syndrome (Sym-bas et al, 1971). The lateralised blood flow throughan aortic Starr-Edwards ball valve prosthesisimpinging on the cuff of aorta left between theaortic annulus and the prosthetic graft was thoughtin that case to have contributed to aneurysm for-mation. Our patient had no outward manifestationsof Marfan's syndrome and retains his own centralorifice flow valve. His aortic histology suggeststhat he may be at some risk in the future fromdevelopment of an aneurysm of the aortic root buthe should be protected from this complication bythe proximal Teflon wrap inserted at operation.Although at present haemodynamically well com-pensated, the residual aortic regurgitation willcertainly necessitate aortic valve replacement inthe future.

We thank Drs Bray, Croxson, and Beton andMr J F Dark for their advice and help in themanagement of this case.

References

Abbott, M E (1927/8). Coarctation of the aorta of theadult type. American Heart Journal, 3, 574-618.

Collins, J J, and Cohn, L H (1973). Reconstruction ofthe aortic valve. Archives of Surgery, 106, 35-37.

Cossette, R, Davignon, A, and Stanley, P (1969). Rup-ture of the aortic aneurysm in a three-and-a-half-year-old-child with coarctation of the aorta. Can-adian Medical Association Journal, 100, 257-261.

Daily, P 0, Trueblood, H W, Stinson, E B, Wuerflein,R D, and Shumway, N E (1970). Management ofacute aortic dissection. A nnals of Thoracic Sur-gery, 10, 237-247.

Dalen, J E, Alpert, J S, Cohn, L H, Black, H, andCollins, J J (1974). Dissection of the thoracic aorta.Medical or surgical therapy? American Journal ofCardiology, 34, 803-808.

Edwards, J E (1973). Aneurysms of the thoracic aortacomplicating coarctation. Circulation, 48, 195-201.

McKusick, V A, Logue, R B, and Bahnson, H T(1957). Association of aortic valvular disease andcystic medial necrosis of the ascending aorta. Cir-culation, 16, 188-194.

McKusick, V A (1972a). Heritable Disorders of Con-nective Tissue. 4th edn. C V Mosby, St Louis.

McKusick, V A (1 972b). Association of congenitalbicuspid aortic valve and Erdheims cystic medicalnecrosis. Lancet, 1, 1026-1027.

Nikaidoh, H, Idriss, F S, and Riker, W L (1973).Aortic rupture in children as a complication ofcoarctation of the aorta. Archives of Surgery, 107,838-841.

Probert, W R (1956). Coarctation of the aorta associ-ated with an aneurysm leading into the bronchialtree, treated by aortic resection and grafting. Pro-ceedings of the Royal Society of Medicine, 49,729-731.

Reifenstein, G A, Levine, S A, and Gross, R E (1947).Coarctation of the aorta. American Heart Journal,33, 146-168.

Strong, W W, Moggio, R A, and Stansel, H G (1974).Acute aortic dissection. Twelve year medical andsurgical experience. Journal of Thoracic and Cardio-Vascular Surgery, 68, 816-821.

Symbas, P N, Raizner, A E, Tyras, D H, Hatcher,C R, Inglesby T V, and Baldwin, B J (1971).Aneurysms of all sinuses of Valsalva in patients withMarfan's syndrome. A nnals of Surgery, 174, 902-907.

Wheat, N W, Palmer, R F, Bartley, T D, and Seel-man, R C (1965). Treatment of dissecting aneurysmsof the aorta without surgery. Journal of Thoracicand Cardia-Vascular Surgery, 50, 364-373.

Requests for reprints to: Robert A M Lawson,FRCS, Cardio-Thoracic Unit, Wythenshawe Hospital,Manchester.

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