Ann Thorac Surg CASE REPORT SANTORO ET AL 1019 1995;59:1019-21 OPERATION IN ISOLATED ATRIAL INVERSION

of acute dissections have all been reported in the litera- ture. McCarthy and associates [3], in 1972, reported a case of acute dissection of the aorta in which the dissection extended through a patent ductus arteriosus, with ulti- mate fistulization to the pulmonary artery. Keenan [4] and Glanz [2] and their colleagues have also observed such fistulas between the aorta and pulmonary artery occurring as the result of acute aortic dissection. Perry- man and Gay [5] reported the only case of an aortic dissection fistulizing to the right ventricle. Rupture of an acute aortic dissection into the right atr ium with fistula formation, found at autopsy, was first reported by Kuipers and Schatz [6] in 1963.

Aortopulmonary, aorta-right atrial, and aorta-right ventricular fistulas all produce left-to-right shunt. Imag- ing plays an important role in the diagnosis of these fistulas. In the past, these fistulas were most frequently diagnosed by aortography and cardiac catheterization, if discovered before the pat ient 's death. Many more, how- ever, were diagnosed post mortem. Echocardiography is a commonly used imaging technique that has become an important tool in the diagnosis of aortic dissection. Two- dimensional echocardiography has the capacity to visu- alize aortic dissections very well [7]. If on physical exam- ination one suspects the presence of a fistula, Doppler echocardiography can be used to further visualize the flow from one compartment to another. It also has the advantage of being able to evaluate left ventricular func- tion and to determine whether there is aortic insuffi- ciency [8]. Veerbeek and associates [9] used transthoracic Doppler echocardiography in the diagnosis of an aorto- pulmonary fistula. This was followed by aortography, which confirmed the diagnosis.

In summary, although aortopulmonary, aorta-atrial, and aorta-ventricular fistulas are uncommon in the set- ting of acute aortic dissections, these complications can be diagnosed preoperatively as long as they are kept in the differential diagnosis whenever patients present with the classic symptoms of left-to-right shunt. One must use all the information and imaging techniques at hand to ensure a t imely diagnosis. Echocardiography has been shown to be an excellent technique for visualizing these complications. However, both magnetic resonance imag- ing and aortography failed to visualize the aortic dissec- tion in our patient. This was probably due to the short segment of aorta involved in the dissection and run-off into the pu lmonary circulation. Aortgraphy did reveal the fistulization with left-to-right shunting, but the find- ings led to the incorrect diagnosis of a ruptured aneu- rysm of the sinus of Valsalva.

R e f e r e n c e s

1. Boyd LJ. A study of four thousand reported cases of aneurysm of the thoracic aorta. Am J Med Sci 1924;168:654-63.

2. Glanz S, Gordon DH, Shah N, Jaffe B, Griepp R. Unusual manifestations of aortic dissection. Cardiovasc Intervent Ra- diol 1982;5:292-5.

3. McCarthy C, Dickson GH, Besterman EMM, Bromley LL, Thompson AE. Aortic dissection, with rupture through duc- tus arteriosus into pulmonary artery. Br Heart J 1972;34: 428 -30.

4. Keenan DJM, Kieso HA, Johnson AM, Ross JK. Acquired aorto-pulmonary fistula: case report. Thorac Cardiovasc Surg 1984;32:190-2.

5. Perryman RA, Gay WA. Rupture of dissecting thoracic aortic

aneurysm into the right ventricle. Am J Cardiol 1972;30: 277-81.

6. Kuipers FM, Schatz IJ. Prognosis in dissecting aneurysm of the aorta. Circulation 1963;27:658-61.

7. Granato JE, Dee P, Gibson RS. Utility of two-dimensional echocardiography in suspected aortic dissection. Am J Car- diol 1985;56:123-9.

8. Dagli SV, Nanda NC, Roitman D, et al. Evaluation of aortic dissection by color-flow mapping. Am J Cardiol 1985;56: 497-8.

9. Veerbeek AG, Van Der Wieken LR, Schuilenburg RM, Blo- emendaal K. Acquired aorto-pulmonary fistula in acute dis- section. Eur Heart J 1992;13:713-5.

Isolated Atrial Inversion in Situs Inversus: A Rare Anatomic Arrangement Giuseppe Santoro, MD, Paolo Masiello, MD, Rosario

Farina, MD, Cesare Baldi, MD, Leonardo Di Leo, MD, and Giuseppe Di Benedetto, MD

Divisions of Cardiac Surgery and Cardiology, Ospedale S. Leonardo, Salerno, Italy

Isolated atrial inversion in situs inversus is a rare con- genital cardiac malformation. Its phys io logy resembles transposition of great vessels , and the best option for its surgical treatment is the atrial switch operation. In this article, w e present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successful ly treated at 8 months of age by a Senning procedure.

(Ann Thorac Surg 1995;59:1019-21)

O ne of the fascinations of the congenital cardiac malformations is that the heart can show virtually

any possible connection or relationship between its main segments (ie, atria, ventricles, and great arteries). One of the rarest combinations is that in which a discordant atrioventricular connection accompanies a concordant ventriculoarterial connection with normally related great arteries. Based on the spatial relationship between the great arteries, "isolated atrial inversion" {SLI} or {IDS} and "isolated ventricular inversion" {SLS} or {IDI} have been dist inguished [1]. This article describes an exceed- ingly rare case of isolated atrial inversion in concordance with visceral situs inversus and reviews the literature about this issue.

A 1-month-old infant was admitted to our hospital be- cause of mild cyanosis since birth. Physical examination revealed a 2/6 systolic m u r m u r at the second left inter- costal space. The liver edge was 2 cm below the left costal margin. Chest roentgenogram showed levocardia and visceral situs inversus (Fig 1). Electrocardiography

Accepted for publication Aug 23, 1994.

Address reprint requests to Dr Di Benedetto, Division of Cardiac Surgery, Ospedale "S. Leonardo," Via S. Leonardo 1, 84100 Salerno, Italy.

© 1995 by The Society of Thoracic Surgeons 0003-4975/95/$9.50 0003-4975(94)00745-S

1020 CASE REPORT SANTORO ET AL Ann Thorac Surg OPERATION IN ISOLATED ATRIAL INVERSION 1995;59:1019-21

on the ventr iculoarter ial re la t ionships [1]. Its anatomic pa t te rn is character ized by discordant atr ioventr icular connection, normal r ight/ left ventr icular relat ionship, concordant ventr iculoarter ial connection, and normal ly re la ted great arteries. Few cases of this anomalous ana- tomic a r rangement have been repor ted, some of which had some degree of heterotaxy [2-4], whereas a few others were in visceroatrial situs solitus. To date, only 4 cases of this anomaly in situs inversus have been re- por ted [5-8]. As expected, isolated atrial inversion shows a circulatory pa t te rn resembl ing classic t ransposi t ion of the great arteries with intercirculatory mixing at the atrial level through an atrial septal defect. Nevertheless , its physiologic and anatomic surgical repai r can be achieved by an atrial switch procedure, by which the morpholog- ically left ventricle serves the systemic circulation and the

Fig 1. Chest roentgenogram in anteroposterior view.

showed a clockwise deviat ion of the P-wave axis with a physiologic sequence of ventr icular depolar izat ion. Ab- dominal u l t rasound examinat ion showed a normal ly formed r ight -s ided single spleen. Bidimensional echocar- d iography and angiocard iography clarified the diagnosis. Systemic venous b lood dra ined through a lef t -s ided inferior vena cava and a left super ior vena cava into a l e f t - s ided morpho log i ca l l y r ight a t r ium. It e n t e r e d through a mitral valve a lef t -s ided morphological ly left ventricle. A r ight-s ided, morphological ly left a t r ium re- ceived all pu lmonary veins and entered through a tricus- p id valve a r ight -s ided morphological ly right ventricle. Right and left ventricles showed topology and relat ion- ship usual for situs solitus. Concordant ventr iculoarter ial connection, normal ly re la ted great arteries, and right aortic arch were found (Fig 2). A modera te - s ized atrial septal defect a l lowed intercirculatory mixing. Systemic arterial saturat ion was 81%.

As the baby grew up duly, a corrective operat ion was pe r fo rmed successfully when the pat ient was 8 months of age. After a med ian sternotomy, aortic cannulat ion was per formed in the usual manner . Venous cannulat ion of the hear t was achieved easily by lifting the hear t from the apex and placing the cannula in the inferior vena cava, after the super ior vena cava had been cannulated. A " 'specular" Senning procedure then was performed.

Postoperat ive course was uneventful. At discharge Holter e lec t rocardiography and echocard iography did not show any significant anomaly.

C o m m e n t

Hearts with discordant atr ioventr icular and concordant ventr iculoarter ial connections from an anatomical ly het- e rogeneous but physiological ly homogeneous entity in which the blood circulation resembles classic t ransposi- tion of the great arteries. Isolated atrial inversion ({SLI} or {IDS}) is a rare condit ion that may be d is t inguished from the isolated ventr icular inversion ({SLS} or {IDI}) based

A

B

Fig 2. Right (A) and left (B) ventriculograms in anteroposterior views, showing ventriculoarterial relationships.

Ann Thorac Surg CASE REPORT FEATHERSTONE ET AL 1021 1995;59:1021-2 ESOPHAGEAL BLEEDING IN CIVlV ESOPHAG1TIS

morphological ly right ventricle serves the pu lmonary circulation [7]. In our patient, a Senning procedure was chosen to avoid using prosthetic material .

In conclusion, a precise segmenta l diagnost ic approach by echocard iography and angiocard iography is manda- tory to define this rare anatomic a r rangement and to make easier appropr ia te surgical managemen t of these patients.

References

1. Pasquini L, Sanders SP, Parness I, et al. Echocardiographic and anatomic findings in atrioventricular discordance with ventriculoarterial concordance. Am J Cardio11988;62:1256-62.

2. Akita H, Matsouka S, Kuroda Y. A rare case of isolated atrial discordance with primary atrial situs inversus. Tokushima J Exp Med 1993;40:113-7.

3. Leijala MA, Lincoln CR, Shinebourn EA, Nellen L. A rare congenital cardiac malformation with situs inversus and dis- cordant atrioventricular and concordant ventriculoarterial connections: diagnosis and surgical treatment. Am Heart J 1981;101:355-6.

4. Clarkson PM, Brandt PWT, Barratt-Boyes BG, Neutze JM. Isolated atrial inversion. Visceral situs solitus, visceroatrial discordance, discordant ventricular d-loop without transpo- sition, dextrocardia: diagnosis and surgical correction. Am J Cardiol 1972;29:877-81.

5. Espino-Vela J, de la Cruz MV, Munoz-Castellanos L, Plaza L, Attie F. Ventricular inversion without transposition of the great vessels in situs inversus. Br Heart J 1970;37:443-50.

6. Squarcia U, Ritter DG, Kincaid OW. Dextrocardia: anglo- graphic study and classification. Am J Cardiol 1973;32:965-77.

7. Fox LS, Kirklin JW, Pacifico AD, Waldo AL, Bargeron LM. Intracardiac repair of cardiac malformations with atrioven- tricular discordance. Circulation 1976;54:123-7.

8. Calcaterra G, Anderson RH, Lau KC, Shinebourne EA. Dex- trocardia: value of segmental analysis in its categorization. Br Heart J 1979;32:497-507.

Massive Esophageal Bleeding in Achalasia Complicated by Cytomegalovirus Esophagitis Roche J. Featherstone, MD, L. Gabrie l Camero, MD, Riad Khatib, MD, Daniel Snower, MD, and Praveena Mungara, MD

St. John Hospital and Medical Center, Detroit, Michigan

A 69-year-old man with achalasia who had received cytoxan and prednisone over a 6-week period for pre- sumed Wegener's granulomatosis presented with mas- sive esophageal bleeding. He did not respond to aggressive medical management, and an emergent esophagectomy was performed. Histologic examination revealed exten- sive cytomegalovirus esophagitis. He had a long but progressive hospital course and was discharged I month after admission. This case illustrates that cytomegalovi- rus esophagitis may cause massive hemorrhage that ne- cessitates surgical intervention.

(Ann Thorac Surg 1995;59:1021-2)

Accepted for publication Aug 20, 1994.

Address reprint requests to Dr Featherstone, St. John Hospital and Medical Center, 22101 Moross Rd, Detroit, MI 48236.

T he patient , a 69-year-old man, p resen ted to our Emergency Depar tmen t with hematemesis . His past

medical history was significant for achalasia of greater than 10 years" durat ion. Six weeks before this admiss ion he was noted to have pers is tent proteinuria; a k idney biopsy was performed, the results of which were thought to be consistent with Wegener ' s granulomatosis . He was s tar ted on a regimen of p rednisone (20 mg every 6 hours) and eyc lophosphamide (50 mg every 12 hours). One week after beginning therapy, upper gastrointest inal b leed ing deve loped that resolved spontaneously . Endoscopy at that t ime revealed no active bleeding, and he was t reated with rani t idine (150 mg every 12 hours). On this admis- sion b leed ing was massive and his hemoglobin level fell to 8.0 gldL.

He was admi t ted to the surgical intensive care unit for resuscitation. Esophagogas t roscopy per formed at bed- side revealed profuse active b leed ing from the mid- esophagus that p rec luded a thorough assessment of the esophagus . The s tomach could not be visualized. At- tempts to control the b leed ing with int ravenous pi tressin and bal loon t amponade with a Sengstaken-Blakemore tube were unsuccessful. The b leeding was massive, and after mul t ip le b lood transfusions, emergency surgical intervent ion was performed.

The pat ient was taken to the opera t ing room immedi - ately and a right posterolateral thoracotomy was per- formed. A huge, purpl ish esophagus, secondary to ve- nous congestion, and a large b loody pleural effusion were found. Dissection of the esophagus was cumbersome due to diffuse per iesophagea l adhes ions and scarring. These areas b led easily as the dissection was performed. The aortic branches to the esophagus were large and friable, as were the veins. This led to significant, cont inuous bleeding. Nevertheless , the dissection cont inued in spite of hypotens ion into the 60 m m Hg systolic range, unti l the intrathoracic esophagus was excised. At this point, the b leed ing s lowed and the pat ient ' s condit ion stabi- l ized with a systolic b lood pressure of 120 to 130 m m Hg. The options of an esophagos tomy and gas t ros tomy ver- sus complet ion of gastric mobil izat ion with esophagogas- t ros tomy were considered. Because the pat ient ' s condi- tion remained stable, we elected to proceed with the gastric mobil izat ion and esophagogast ros tomy, which the pat ient tolerated well.

Postoperatively, b lood cont inued to ooze from the chest tube, and 24 hours later the pat ient was taken for a repea t thoracotorny. This explorat ion revealed b leed ing from a vessel involved in the gastric mobil izat ion. Bleed- ing was control led and the pat ient was re turned to the intensive care unit. The pat ient r emained in the intensive care unit for approximate ly 4 weeks. He received 31 units of packed red blood cells and 12 units of fresh frozen plasma. Pathologic s tudy showed the esophageal wall to be marked ly thickened. The mucosal surface had multi- ple areas of small ulceration. Histologic examinat ion confirmed the marked hyper t rophy of the muscularis propr ia consistent with achalasia. The areas of ulcerat ion revealed a f ibropurulent exudate with acute inf lamma- tion and granulat ion tissue (Fig 1) reaching to the super-

© 1995 by The Society of Thoracic Surgeons 0003-4975/95/$9.50 0003-4975(94)00744-R