*****AJR. AMERICAN JOURNAL OF ROENTGENOLOGY*****

(REFERENCE 1 OF 41) 82156702

Tonkin IL Tonkin AK Visceroatrial situs abnormalities: sonographic and computed tomographic appearance.

In: AJR Am J Roentgenol (1982 Mar) 138(3):509-15

ISSN: 0361-803X

Twenty patients with visceroatrial situs abnormalities were examined by sonography or computed tomography (CT). Eighteen patients underwent cardiac catheterization. Left isomerism (polysplenia syndrome) was found in seven patients, right isomerism (asplenia syndrome) in eight, and total situs inversus in five. The abdominal features of polysplenia include interruption of the inferior vena cava with azygous/hemiazygous continuation (100%) and multiple splenules. The diagnostic signs of asplenia include an inferior vena cava and aorta that course together on the same side of the spine with inferior vena cava-atrial communication (100%). In each case, the key differential feature relates to the major abdominal vessels. Sonography or CT examination in cases of situs ambiguus may reveal diagnostic features that can be used with radiographic signs to accurately diagnose the visceroatrial situs.

*****AMERICAN FAMILY PHYSICIAN*****

(REFERENCE 2 OF 41) 91281646

Kinney TB DeLuca SA Kartagener's syndrome.

In: Am Fam Physician (1991 Jul) 44(1):133-4

ISSN: 0002-838X

Kartagener's syndrome is characterized by situs inversus, chronic paranasal sinusitis and bronchiectasis. The pathogenesis of this rare condition is an ultrastructural defect that results in immotile cilia. Radiologic features may include findings consistent with bronchiectasis.

Institutional address: Department of Radiology Massachusetts General Hospital Boston.

*****AMERICAN JOURNAL OF CARDIOLOGY*****

(REFERENCE 3 OF 41) 77263976

Hazan E Baillot F Rey C Dupuis C Isolated ventricular discordance and complete atrioventricular canal in situs inversus. Report of successful surgical repair.

In: Am J Cardiol (1977 Sep) 40(3):463-6

ISSN: 0002-9149

Isolated ventricular discordance is a very rare malformation, most often lethal in the first months of life. A patient with this lesion survived to age 9 years in spite of the coexistence of a complete form of atrioventricular (A-V) canal. Surgical repair with a Mustard procedure was associated with correction of the endocardial cushion defect. Situs inversus itself caused no problem. Although permanent complete A-V block occurred, the result has been satisfactory so far. This case is thought to be the first successful correction of this malformation.

(REFERENCE 4 OF 41) 98122456

Ruscazio M Van Praagh S Marrass AR Catani G Iliceto S Van Praagh R Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities [see comments]

In: Am J Cardiol (1998 Jan 1) 81(1):111-6

ISSN: 0002-9149

We present the clinical and postmortem findings of the first photographically documented case of asplenia and interrupted inferior vena cava and the anatomic findings of 5 previously reported cases. A brief review of the various hereditary patterns of visceral situs abnormalities suggests that, at least in some cases, the asplenia and polysplenia syndromes are etiologically and pathogenetically interrelated.

Comment in: Am J Cardiol 1998 Jun 15;81(12):1522

Institutional address: Instituto di Cardiologia Universita'degli Studi di Cagliari Cagliari Sardinia Italy.

(REFERENCE 5 OF 41) 94197101

Phoon CK Neill CA Asplenia syndrome: insight into embryology through an analysis of cardiac and extracardiac anomalies [see comments]

In: Am J Cardiol (1994 Mar 15) 73(8):581-7

ISSN: 0002-9149

Asplenia syndrome is characterized by complex congenital heart defects, asplenia and abdominal heterotaxy. Recent interest in the syndrome has been increased by new knowledge arising from animal models and by continuing improvements in surgical outcome in childhood. To further elucidate the embryologic timing and mechanisms of the asplenia syndrome, 32 necropsy cases were reviewed and 487 published autopsy cases were reanalyzed at the hospital. The most common congenital heart defects were atrial septal defects, common atrioventricular canals and conotruncal anomalies. With use of current information on the timing of normal development, it was hypothesized that most defects originate at Streeter Horizon XIII; patients averaged 3.2 Horizon XIII defects, more than at any other stage. Distribution was unimodal. Extracardiac anomalies also exhibited a developmental spectrum. Because the normal spleen develops by Horizon XIII, asplenia, the sine qua non of the syndrome, originates then or earlier. Abnormal pulmonary lobation occurred in 80% of cases, with right isomerism occurring most often; pulmonary branching asymmetry also originates at or before Horizon XIII. Abdominal heterotaxy occurred in 72% of cases, but the timing of origin is unclear. Anomalies of other systems, including genitourinary, musculoskeletal, endocrine, and nervous systems, develop later (typically XV to XXIII); specific anomalies were less frequent, although much more prevalent than in the general population. It is concluded that asplenia syndrome is a focal developmental disturbance in laterality which occurs primarily at Horizon XIII.

Comment in: Am J Cardiol 1994 Nov 15;74(10):1079

Comment in: Am J Cardiol 1995 Feb 1;75(4):317

Institutional address: Division of Pediatric Cardiology Johns Hopkins University School of Medicine Baltimore Maryland.

(REFERENCE 6 OF 41) 94070653

Levine JC Walsh EP Saul JP Radiofrequency ablation of accessory pathways associated with congenital heart disease including heterotaxy syndrome.

In: Am J Cardiol (1993 Sep 15) 72(9):689-93

ISSN: 0002-9149

Congenital heart disease complicates the management of most accessory pathway-mediated tachycardias and also increases the challenge of radiofrequency ablation. Since 1990, radiofrequency ablation of accessory atrioventricular (AV) pathways has been attempted in 10 patients (age range 3.5 months to 30 years) with congenital heart disease: Ebstein's anomaly (n = 5), heterotaxy with AV discordance (n = 3), tetralogy of Fallot (n = 1) and total anomalous pulmonary venous return (n = 1). Eight patients had manifest Wolff-Parkinson- White syndrome and 2 had concealed pathways. Five patients had multiple pathways including 4 of the 5 with Ebstein's anomaly. Of 16 pathways total, 15 were associated with the tricuspid valve including all pathways in the patients with Ebstein's anomaly and heterotaxy. The His bundle area was identified in all patients and involved an anterior AV node in 2 of 3 with heterotaxy. Ablation was performed on the atrial side of the AV ring in all cases. No instance of AV block was encountered. Complete success was achieved in 6 patients including the 3 with heterotaxy. In 2 patients, manifest preexcitation was eliminated and clinical symptoms were greatly modified. The procedure was transiently successful in 1 patient who later had surgical interruption of the accessory pathway during tetralogy of Fallot repair. Ablation was unsuccessful in 1 patient. Thus, the overall success was 80%.

Institutional address: Department of Cardiology Children's Hospital Boston Massachusetts 02115.

*****AMERICAN JOURNAL OF THE MEDICAL SCIENCES*****

(REFERENCE 7 OF 41) 76181927

Fishman JE Spaier AH Cohen MM Familial dextrocardia, divergent strabismus and situs inversus of optic disc.

In: Am J Med Sci (1976 Mar-Apr) 271(2):225-31

ISSN: 0002-9629

A family is described in which three of four siblings have major congenital cardiac defects in association with ocular abnormalities. The eldest sibling has isolated dextrocardia and the second has total situs inversus. The fourth child has both atrial and ventricular septal defects, with pulmonary hypertension and right ventricular hypertrophy. The mother and third sibling have normal hearts but exhibit a number of ocular defects. The principal ocular anomalies demonstrated in this family are divergent strabismus, bilateral situs inversus of the optic disc, and myopia. The hereditary aspect of these multiple findings is discussed.

*****AMERICAN JOURNAL OF ROENTGENOLOGY, RADIUM THERAPY AND NUCLEAR*****

(REFERENCE 8 OF 41) 76085677

Tegtmeyer CJ Hust FS Keats TE Arteriographic manifestations of abdominal situs inversus.

In: Am J Roentgenol Radium Ther Nucl Med (1975 Oct) 125(2):427-30

ISSN: 0002-9580

The arteriographic findings are described and the embryology is discussed in a rare case of situs inversus of the abdominal viscera combined with reversed rotation of the bowel. The arteriogram correctly localized the precise site of hemorrhage in this perplexing case, and a complex surgical problem was simplified.

*****ANNALS OF SURGERY*****

(REFERENCE 9 OF 41) 95344277

Farmer DG Shaked A Olthoff KM Imagawa DK Millis JM Busuttil RW Evaluation, operative management, and outcome after liver transplantation in children with biliary atresia and situs inversus.

In: Ann Surg (1995 Jul) 222(1):47-50

ISSN: 0003-4932

INTRODUCTION: Biliary atresia, a common indication for liver transplantation, can be associated with situs inversus. Our experience with liver transplantation in children (n = 6) was reviewed retrospectively. PATIENTS AND METHODS: Preoperative duplex sonography, computerized tomography, and visceral angiography were obtained. Vascular anomalies identified included preduodenal portal vein (6/6), interrupted inferior vena cava (5/6), and aberrant hepatic artery (4/6). RESULTS: The liver graft was placed in a midline position. Venous continuity was achieved by donor suprahepatic inferior vena cava to recipient hepatic cloaca and direct end-to-end portal anastomosis. The donor infrahepatic inferior vena cava was oversewn. Arterial continuity was restored using either a direct branch-patch anastomosis (3/6) or a supraceliac aortic interposition graft (3/6). In retrospect, preoperative diagnostic work-up was noncontributory and outcome was not complicated by pre- existing situs inversus. CONCLUSION: Situs inversus in liver recipients requires operative technical modifications, but does not change outcome. Furthermore, extensive preoperative work-up should be avoided.

Institutional address: Dumont UCLA Transplant Center Department of Surgery UCLA School of Medicine 90024 USA.

*****ANNALS OF THORACIC SURGERY*****

(REFERENCE 10 OF 41) 85045682

Watson DC Shapiro SR Midgley FM Scott LP Surgical approach to corrected transposition of the great vessels and situs inversus [I, D, D] with ventricular septal defect and systemic atrioventricular valve regurgitation.

In: Ann Thorac Surg (1984 Nov) 38(5):520-1

ISSN: 0003-4975

Successful surgical repair of a 4-year-old boy with situs inversus [I, D, D] and corrected transposition of the great vessels (TGV) as well as hemodynamically significant ventricular septal defect (VSD), systemic atrioventricular (tricuspid) valve regurgitation, and atrial septal defect is described. Unique technical aspects of VSD and tricuspid valve repair in association with corrected TGV and situs solitus or inversus are discussed and clarified.

(REFERENCE 11 OF 41) 95374109

Rubay JE d'Udekem Y Sluysmans T Ponlot R Jacquet L de Leval MR Orthotopic heart transplantation in situs inversus.

In: Ann Thorac Surg (1995 Aug) 60(2):460-2

ISSN: 0003-4975

The case of a successful orthotopic heart transplantation for complete situs inversus with double-inlet left ventricle and anomalies of the systemic venous return is reported. A piece of aortic homograft and a composite conduit made of the recipient right atrium and pericardium were used to connect, respectively, the left superior vena cava and the hepatic veins to the right-sided atrium of the donor heart.

Institutional address: Department of Cardiovascular and Thoracic Surgery Cliniques Universitaires St-Luc Brussels Belgium.

(REFERENCE 12 OF 41) 95321819

Michler RE Sandhu AA Novel approach for orthotopic heart transplantation in visceroatrial situs inversus.

In: Ann Thorac Surg (1995 Jul) 60(1):194-7

ISSN: 0003-4975

Orthotopic heart transplantation was performed successfully in a 7- year-old girl with visceroatrial situs inversus. Creation of two autologous left-sided atrial tissue baffles tunneled the left superior vena cava and inferior vena cava to the right of the pulmonary veins. The reconstructed caval tunnels remain widely patent more than 4 years after transplantation. The ability of autologous tissue to grow with the patient has distinct advantages in the pediatric transplant population.

Institutional address: Department of Surgery Columbia University College of Physicians and Surgeons New York New York USA.

(REFERENCE 13 OF 41) 95209423

Santoro G Masiello P Farina R Baldi C Di Leo L Di Benedetto G Isolated atrial inversion in situs inversus: a rare anatomic arrangement.

In: Ann Thorac Surg (1995 Apr) 59(4):1019-21

ISSN: 0003-4975

Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.

Institutional address: Division of Cardiac Surgery Ospedale S. Leonardo Salerno Italy.

(REFERENCE 14 OF 41) 95031472

Mentzer SJ Aranki SF Reilly JJ DeCamp MM Hartigan P O'Donnell W Sugarbaker DJ Single-lung transplantation in situs inversus.

In: Ann Thorac Surg (1994 Oct) 58(4):1176-8

ISSN: 0003-4975

Situs inversus totalis is a rare anatomic condition characterized by the mirror-imaged arrangement of asymmetric thoracic and abdominal organs. Although associated cardiopulmonary disease is uncommon, end- stage lung disease can develop in patients with situs inversus, necessitating transplantation. In this report, we describe a 30-year- old patient with situs inversus totalis and end-stage pulmonary hypertension who underwent successful orthotopic left lung transplantation.

Institutional address: Department of Surgery Brigham and Women's Hospital Harvard Medical School

Boston Massachusetts 02115.

(REFERENCE 15 OF 41) 95031471

Parry AJ O'Fiesh J Wallwork J Large SR Heart-lung transplantation in situs inversus and chest wall deformity.

In: Ann Thorac Surg (1994 Oct) 58(4):1174-6

ISSN: 0003-4975

Heart-lung transplantation in the presence of complex congenital heart disease including situs inversus and significant chest wall deformity can be accomplished successfully. However, the postoperative course is apt to be prolonged because of mechanical respiratory problems, which will respond to a protocol of weaning and nutritional supplementation.

Institutional address: Transplant Unit Papworth Hospital Cambridge England.

(REFERENCE 16 OF 41) 96266262

Rabago G Copeland JG 3rd Rosapepe F Tsen AC Arzouman DA Arabia FA Sethi GK Heart-lung transplantation in situs inversus.

In: Ann Thorac Surg (1996 Jul) 62(1):296-8

ISSN: 0003-4975

Reconstruction was accomplished in a 2 heart-lung recipients with situs inversus resulting in a left-sided systemic venous atrium. We created a large common atrium that was closed on the left side, leaving an atrial cuff on the inferior right quadrant. To this we anastomosed the donor right atrium, which had been opened laterally between the cavae. This resulted in some clockwise rotation of the ventricles and anterior positioning of the apex. The right pulmonary veins passed superior to the atrial anastomosis and posterior to the donor right atrium. Cardiopulmonary function was excellent in both cases.

Institutional address: Department of Cardiovascular and Thoracic Surgery University of Arizona Tucson USA.

(REFERENCE 17 OF 41) 86102325

Baudet EM Hafez A Choussat A Roques X Isolated ventricular inversion with situs solitus: successful

surgical repair.

In: Ann Thorac Surg (1986 Jan) 41(1):91-4

ISSN: 0003-4975

A 7-month-old infant with ventricular inversion without transposition of the great arteries in situs solitus, associated with a ventricular septal defect, is reported. When the infant was 13 months of age, successful surgical repair was undertaken by performing an intraatrial transposition of venous return using the Senning procedure and by closing the ventricular septal defect with a patch. The postoperative course was uneventful except for a well-tolerated complete heart block. No systemic valve incompetence appeared during the 53 months of actual follow-up. A complete review of the literature suggests that this is the sixth reported instance of successful repair. Injury of the abnormally located conduction tissue is regarded as the most threatening complication.

*****ARCHIVES OF SURGERY*****

(REFERENCE 18 OF 41) 92028373

Organ BC Skandalakis LJ Gray SW Skandalakis JE Cancer of bile duct with situs inversus.

In: Arch Surg (1991 Sep) 126(9):1150-3

ISSN: 0004-0010

We present a case of adenocarcinoma of the distal common bile duct in a 68-year-old woman with total situs inversus. Endoscopic retrograde cholangiopancreatography prior to surgery demonstrated segmental obstruction of the distal common bile duct. Obstructive jaundice was observed and the results of liver function studies were abnormal. All biopsy specimens obtained in the operating room were negative. The case met all the criteria of Child and Frey, but at the end of the procedure the tumor was found to involve the superior mesenteric vein, a factor not appreciated during the operative evaluation of the patient. The situs inversus did not produce any technical problems. The patient did not have any surgical complications, but she died 18 months later of the cancer.

Institutional address: Thalia and Michael Carlos Center for Surgical Anatomy and Technique Atlanta Ga.

*****BRITISH HEART JOURNAL*****

(REFERENCE 19 OF 41) 82231745

Huhta JC Smallhorn JF Macartney FJ Two dimensional echocardiographic diagnosis of situs.

In: Br Heart J (1982 Aug) 48(2):97-108

ISSN: 0007-0769

At present there is no reliable method of recognising atrial isomerism by two dimensional echocardiography. We therefore used two dimensional echocardiography to examine 158 patients including 25 with atrial isomerism and four with situs inversus. Particular attention was paid to the short and long axis subcostal scans of the abdomen. Using the position of the inferior vena cava and the aorta with respect to the spine it was possible to separate those with situs solitus from the others. Two false positives for abnormal situs had exomphalos. In situs solitus the aorta lay to the left of the spine and the inferior vena cava lay to the right. One patient with situs solitus and azygos continuation of the inferior vena cava also had inferior vena cava to right atrial connection. In the four patients with situs inversus the mirror image of the normal pattern was present. In nine patients with right isomerism the inferior vena cava and aorta ran together on one or other side of the spine. The inferior vena cava, anterior to the aorta at the level of the diaphragm, received at least the right hepatic veins (normal or partial anomalous hepatic venous connection). Of the 16 patients with left isomerism, 14 had azygos continuation of the inferior vena cava which was visualised posterior to the aorta in all but two. All patients with left isomerism had total anomalous hepatic venous connection to one or both atria via one or two separate veins. Two dimensional echocardiography therefore provides the means of detecting abnormal atrial situs and of diagnosing right or left isomerism in the great majority of patients, if not all.

(REFERENCE 20 OF 41) 78103674

Wilkinson JL Smith A Lincoln C Anderson RH Conducting tissues in congenitally corrected transposition with situs inversus.

In: Br Heart J (1978 Jan) 40(1):41-8

ISSN: 0007-0769

Three cases of congenitally corrected transposition in situs inversus individuals were characterised by visceroatrial situs inversus, atrioventricular discordance, and ventiruloarterial discordance: one case was studied clinically, and the other 2 were necropsy specimens. The disposition of the atrioventricular conducting tissues was established in each case, in the living patient by intraoperative mapping, and in the necropsy specimens by histopathological investigation. In all, the connecting atrioventricular bundle arose from a normally situated posterior atrioventricular node, normally related to the landmarks of the atrial septum. Though anterior nodes were identified in the necropsy specimens, as reported in congenitally corrected transposition in situs individuals, they differed from those in the latter situation in that they made no atrioventricular connection. These findings have obvious surgical importance. It is suggested that the posterior connection is related to the good septal alignment in these cases which lacked significant septal defects. Posterior connections are not necessarily present in all situs inversus individuals with corrected transposition, particularly when there are malalignment ventricular septal defects: further studies are required in such cases.

*****BRITISH JOURNAL OF SURGERY*****

(REFERENCE 21 OF 41) 95268781

Watson CJ Rasmussen A Jamieson NV Friend PJ Johnston PS Barnes ND Calne RY Liver transplantation in patients with situs inversus.

In: Br J Surg (1995 Feb) 82(2):242-5

ISSN: 0007-1323

Seven patients with situs inversus abdominis and one with situs inversus totalis underwent liver transplantation; all are alive at follow-up of between 7 months and 5 years. Two patients required retransplantation within the first 3 weeks (for primary non-function and thrombotic infarction). Seven had additional abnormalities associated with the polysplenia-biliary atresia syndrome. Liver transplantation in these patients involved selection of relatively small donor organs or use of reduced-size grafts. Delayed abdominal wall closure was necessary in two patients and all required a modification of the 'piggy-back' technique of suprahepatic vena caval anastomosis to overcome recipient venous anomalies. Biliary drainage by Roux-en-Y choledochojejunostomy was the preferred technique. Although technically challenging, situs inversus is not a contraindication to liver transplantation and patients should expect full recovery.

Institutional address: Department of Surgery Addenbrooke's Hospital Cambridge UK.

*****CHEST*****

(REFERENCE 22 OF 41) 76256597

Kleinfeld M Rozanski JJ Brumlik JV Situs inversus, subaortic and subpulmonic stenosis, ventricular septal defect, and single coronary artery.

In: Chest (1976 Sep) 70(03):391-3

ISSN: 0012-3692

The unusual occurrence of situs inversus totalis, ventricular septal defect, hypertrophic subaortic and subpulmonic stenosis, and single coronary artery in a 38-year-old man is presented. The clinical course was remarkably mild, as documented by data from 23 years of study including four cardiac catheterizations. At age 35 years, however, syncope, chest pain, and marked elevation of right ventricular pressure prompted complete surgical repair of the left and right ventricular outflow tract obstructions and closure of the septal defect. Three years after surgery the patient continues to lead an active life without symptoms. The unusually mild course can be attributed to the natural banding effects of the subpulmonic stenosis which prevented irreversible pulmonary hypertension.

(REFERENCE 23 OF 41) 76117262

Liem KL ten Veen JH Inferior myocardial infarction in a patient with mirror-image dextrocardia and situs inversus totalis.

In: Chest (1976 Feb) 69(2):239-41

ISSN: 0012-3692

A case of inferior wall myocardial infarction in a patient with mirror-image dextrocardia and situs inversus totalis is presented. The clinical and electrocardiographic findings are discussed.

(REFERENCE 24 OF 41) 77137575

Wells DE Befeler B Situs inversus totalis associated with subaortic and subpulmonic stenosis.

In: Chest (1975 Jan) 67(1):101-3

ISSN: 0012-3692

The unusual occurrence of total situs inversus and idiopathic hypertrophic subaortic stenosis with the demonstration of right and left heart dynamic obstruction in one patient is presented. The fact that the patient was known to have a rare abnormality (total situs inversus) and the presenting symptom being angina pectoris, may have obscured the diagnosis of IHSS and emphasized the value of comprehensive evaluation of patients. The documentation of right ventricular dynamic obstruction, in addition to obstruction of the left side of the heart, was facilitated by the use of simultaneous pressure recordings in the pulmonary artery and in the body of the right ventricle. The angina-like pain was secondary to left ventricular outflow obstruction, rather than to coronary disease.

(REFERENCE 25 OF 41) 89004307

Abensur H Ramires JA Dallan LA Jatene A Right mammary-coronary anastomosis in a patient with situs inversus.

In: Chest (1988 Oct) 94(4):886-7

ISSN: 0012-3692

We report a patient with situs inversus and ischemic heart disease who had myocardial revascularization with anastomosis of the right mammary artery to the anterior descending coronary artery.

Institutional address: Division of Cardiology Instituto do Corac~ao da Universidade de S~ao Paulo Brazil.

*****CIRCULATION*****

(REFERENCE 26 OF 41) 92346844

Seo JW Brown NA Ho SY Anderson RH Abnormal laterality and congenital cardiac anomalies. Relations of visceral and cardiac morphologies in the iv/iv mouse.

In: Circulation (1992 Aug) 86(2):642-50

ISSN: 0009-7322

BACKGROUND. In the management of hearts with deranged laterality, it is essential that the left and right atrial chambers are correctly identified. There are two major approaches, which are based on venous connections or on the morphology of the atrial appendages, and there is no consensus as to which is the most useful. We used the iv/iv mouse mutant, which is known to be pertinent to this problem, to evaluate the relations of cardiac defects with atrial, venous, and

other visceral morphologies. METHODS AND RESULTS. The morphology of the heart and other organs was examined in 275 iv/iv mice using criteria based on abnormal laterality in humans. The arrangement of the atrial appendages was determined by morphological examination of the junction between the appendage and the venous component of the atrium. On this basis, 45.1% of cases were shown to have usual atrial arrangement, 50.2% had mirror imagery, 1.5% had right isomerism, and

3.3% had left isomerism. Every case of atrial isomerism had a cardiac lesion; the morphological types were similar to those seen in human cases. Of cases with either usual or mirror-image arrangement of the appendages, 33.2% had abnormal spleens, but only 3.1% had cardiac defects. Similarly, venous abnormalities were much more common (30.1%) than cardiac defects. CONCLUSIONS. Study results endorse the importance of the morphology of atrial appendages in predicting cardiac abnormalities and point to the marked inconsistency of the arrangement of other organs, including the spleen and the connections of the systemic veins.

Institutional address: Department of Paediatrics National Heart and Lung Institute London UK.

(REFERENCE 27 OF 41) 92069864

Icardo JM Sanchez de Vega MJ Spectrum of heart malformations in mice with situs solitus, situs inversus, and associated visceral heterotaxy.

In: Circulation (1991 Dec) 84(6):2547-58

ISSN: 0009-7322

BACKGROUND. We present a study of the heart malformations found in a collection of mouse fetuses of the iv/iv strain between days 16.5 and 18.5 of gestation. METHODS AND RESULTS. One hundred hearts were serially sectioned and studied by segmental analysis with a light microscope. Forty additional hearts were analyzed with a scanning microscope. Forty percent of the hearts were found to be malformed. The most frequently occurring heart malformations were persistence of the sinus venosus (9%), common atrium (17%), common atrioventricular canal (24%), double-outlet right ventricle (12%), Fallot's tetralogy (8%), and transposition of the great arteries (5%). These malformations do not usually occur in isolation but rather appear in the formation of complex cardiopathies. The most severe and frequent is the combination of persistence of sinus venosus, common atrium, common atrioventricular canal, and double-outlet right ventricle; this is the "bulboventricular heart." The morphology of each lesion, as well as the degree of association, is similar to that found in human hearts with complex cardiopathies. Some of these cardiopathies appear to be directly related to formation of the cardiac loop. The iv/iv mouse appears to constitute an excellent model with which to study the etiology and pathogenesis of complex heart defects in humans. These hearts show a high phenotypic variability in the presentation of heart lesions. From a genetic viewpoint, there is a basic defect--the bulboventricular heart--which can be considered congenital. The other malformations can be considered formes frustes of the defect type. CONCLUSIONS. The iv gene is a developmental gene that affects basic developmental mechanisms. In this regard, heart lesions may not be the primary result of the abnormal gene activity but rather are secondary to defective interactions during cardiac development.

Institutional address: Department of Anatomy and Cell Biology University of Cantabria Santander Spain.

(REFERENCE 28 OF 41) 92035492

Di Donato RM Wernovsky G Jonas RA Mayer JE Jr Keane JF Castaneda AR Corrected transposition in situs inversus. Biventricular repair of associated cardiac anomalies.

In: Circulation (1991 Nov) 84(5 Suppl):III193-9

ISSN: 0009-7322

Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.7 +/- 8.6 yr) underwent repair of associated cardiac anomalies, including ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) in all 13 patients and atrial septal defect (ASD) in 11. Systemic venous anomalies were present in four. Nine patients also had dextrocardia. Patches to close the VSD were placed on the right side of the ventricular septum (through a right [systemic] ventriculotomy) in two patients and on the left side of the ventricular septum in the other 11 via a left ventriculotomy in one or a morphological right (left-sided) atriotomy in 10. The POTO was relieved directly in five patients and bypassed by a left ventricle-

pulmonary artery conduit in the other eight. There was one early death due to cerebral hemorrhage. Two patients required a pacemaker for permanent heart block. Other rhythm disturbances included transient heart block in three patients and late atrial fibrillation in one. All the survivors are asymptomatic as late as 15.5 years after the repair. One patient had conduit revision 10.5 years after the initial operation, two had residual POTO of greater than 40 mm Hg, and none had residual VSD. In conclusion, patients with [I,D,D] C- TGA present with a relatively uniform subset of anatomic lesions, including predominantly ASD, VSD, and POTO.(ABSTRACT TRUNCATED AT 250 WORDS)

Institutional address: Department of Cardiovascular Surgery Children's Hospital Boston MA 02115.

(REFERENCE 29 OF 41) 77160500

Dick M 2nd Van Praagh R Rudd M Folkerth T Castaneda AR Electrophysiologic delineation of the specialized atrioventricular conduction system in two patients with corrected transposition of the great arteries in situs inversus (I,D,D).

In: Circulation (1977 Jun) 55(6):896-900

ISSN: 0009-7322

Electrophysiologic delineation of the atrioventricular conduction system at surgery is described in two patients with corrected transpostion of the great arteries in situs inversus. Intra-atrial electrograms were recorded in one patient from sites immediately adjacent to the coronary sinus located in the left-sided right atrium. The intraventricular portion of the atrioventricular conduction system was identified in both patients along the posterior and inferior margin of the ventricular septal defect, in contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. In contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. The course of the conduction system in the hearts of these two patients and a possible relationship to the cardiac loop and dual origin of the atrioventricular node is discussed. These cases illustrate the usefulness of segmental diagnosis of congenital heart disease and of electrophysiologic identification of the specialized atrioventricular conduction system at surgery.

*****GASTROENTEROLOGY*****

(REFERENCE 30 OF 41) 80025513

Pastakia B Lieberman LM Moodie D Levy J 99mTechnetium pyridoxylidene glutamate imaging in visceral heterotaxy (Ivemark's syndrome).

In: Gastroenterology (1979 Nov) 77(5):1105-8

ISSN: 0016-5085

A two and one-half year old boy with complex congenital heart disease had Howell-Jolly bodies in his blood raising the possibility of the asplenia syndrome. A 99mTc-sulfur colloid liver-spleen scan was reported normal, but the right lobe of the abnormally reversed liver was mistaken for the spleen. The diagnosis of visceral heterotaxy (Ivemark's syndrome) was established after scanning the patient with a new radiopharmaceutical, 99mTechnetium pyridoxylidene glutamate. The agent clearly demonstrated a left-sided gall bladder, and on comparison with the sulfur colloid scan it was established that asplenia was present. Radionuclide imaging with the new generation of hepatobiliary agents is a reliable method to document asplenia and is useful in studying patients with visceral heterotaxy.

*****JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY*****

(REFERENCE 31 OF 41) 92139893

Miralles A Muneretto C Gandjbakhch I Lecompte Y Pavie A Rabago G Bracamonte L Desruennes M Cabrol A Cabrol C Heart-lung transplantation in situs inversus. A case report in a patient with Kartagener's syndrome.

In: J Thorac Cardiovasc Surg (1992 Feb) 103(2):307-13

ISSN: 0022-5223

After a long history of recurrent chronic pulmonary infections in a 25-year-old woman with Kartagener's syndrome, a heart-lung transplantation was performed. A modified surgical procedure was needed to perform transplantation because of the presence of a situs inversus, which is usually associated with bronchiectasis and sinusitis in this congenital syndrome. A large single atrium was created with both the right and left recipient atria used to facilitate anastomosis with the donor's right atrium. The patient was discharged after resolution of early ventilatory complications and is in good condition 8 months after transplantation.

Institutional address: Department of Cardiac Surgery La Pitie Paris France.

(REFERENCE 32 OF 41) 76124130

Stewart S Farnham JD Schreiner B Manning J Complete correction of double-outlet right ventricle with situs inversus, 1-loop, and 1-malposition (I,L,L) with subaortic VSD and pulmonary stenosis.

In: J Thorac Cardiovasc Surg (1976 Jan) 71(1):129-33

ISSN: 0022-5223

The repair a unique case of double-outlet right ventricle is reported. This case illustrates the increasingly difficult problem of developing an appropriate nomenclature in complex congenital heart disease. A classification of double-outlet right ventricle based upon Van Praagh's symbolic terminology is suggested.

(REFERENCE 33 OF 41) 90173358

Doty DB Renlund DG Caputo GR Burton NA Jones KW Cardiac transplantation in situs inversus.

In: J Thorac Cardiovasc Surg (1990 Mar) 99(3):493-9

ISSN: 0022-5223

Transplantation of the heart was successfully performed in a patient with situs inversus of the viscera and atria. Anatomic constants common to all patients allowed the left atrium, pulmonary artery, and aorta to be joined as usual in the transplant operation. Systemic venous reconstruction was the crux of the operation. A composite superior vena cava was reconstructed on the right side from the recipient translocated superior vena cava, donor superior vena cava and innominate vein, and recipient in situ pericardium. The inferior vena cava was rerouted from the left side across the midline to the right through a composite conduit consisting of the recipient night atrium and in situ pericardium over the diaphragm. These reconstructed venous passageways have remained patent and unobstructed for 1 1/2 years after the operation.

Institutional address: Department of Surgery University of Utah School of Medicine Salt Lake City.

(REFERENCE 34 OF 41) 98335622

Vricella LA Razzouk AJ Gundry SR Larsen RL Kuhn MA Bailey LL Heart transplantation in infants and children with situs inversus.

In: J Thorac Cardiovasc Surg (1998 Jul) 116(1):82-9

ISSN: 0022-5223

BACKGROUND: Recipient situs inversus has always represented a technical challenge during heart transplantation. OBJECTIVE: A simplified operative strategy for heart transplantation in a recipient with atrial situs inversus is described. METHODS: Fifteen pediatric recipients with situs inversus accompanying other complex congenital heart disease or dilated cardiomyopathy having "orthotopic" heart allotransplantation in one center, between 1985 and 1997, were reviewed retrospectively. A nearly uniform, simplified technical approach to transplantation was used and is described. RESULTS: Fourteen of these recipients with complex malformations survived the transplantation. Morbidity relating to surgical technique has been limited to partial (n = 2) or complete (n = 1) late obstruction of superior vena caval drainage; each case was managed successfully by interventional cardiologic techniques. Actuarial survival after transplantation compares favorably with that among 290 infants and children with atrial situs solitus who underwent heart transplantation. CONCLUSIONS: Systemic atrial malposition, including situs inversus, does not limit successful heart transplantation by the simplified method described.

Institutional address: Division of Cardiothoracic Surgery Loma Linda University Medical Center and Children's Hospital Calif 92354 USA.

(REFERENCE 35 OF 41) 94301026

Macchiarini P Chapelier A Vouhe P Cerrina J Ladurie FL Parquin F Brenot F Simonneau G Dartevelle P Double lung transplantation in situs inversus with Kartagener's syndrome. Paris-Sud University Lung Transplant Group [see comments]

In: J Thorac Cardiovasc Surg (1994 Jul) 108(1):86-91

ISSN: 0022-5223

En bloc double lung transplantation with bilateral bronchial anastomoses was successfully performed in three patients with complete situs inversus and end-stage Kartagener's syndrome. Dextrocardia was not a technical problem for institution of cardiopulmonary bypass, but a large azygos vein draining the systemic venous return was systematically preserved. The major technical difficulty was restoration of airway continuity, because patients with situs inversus have an inverse direction and length of the main stem bronchi. The right and left main bronchi of the recipients were approached in the aortocaval sinus and transected approximately at 1.5 cm from the carina. The donor right main stem bronchus was divided at its origin and the donor left main stem bronchus was divided proximal to the upper lobe takeoff. The different bronchial angulation was not an obstacle, and airway continuity was reestablished twice with an end-to-end anastomosis and once with a telescopic technique. Because of the midline position of the left atrium and pulmonary artery, the anastomoses with the respective recipient's structures were made as in patients with situs solitus. One patient required a right lower lobectomy because the position of the right side of the heart interfered with lobar expansion. One patient died of obliterative bronchiolitis 36 months after the operation. The remaining two are alive and doing well after 48 and 6 months, respectively.

Comment in: J Thorac Cardiovasc Surg 1994 Nov;108(5):992-3

Institutional address: Department of Thoracic and Vascular Surgery Hopital Marie-Lannelongue Le Plessis Robinson France.

*****JOURNAL OF UROLOGY*****

(REFERENCE 36 OF 41) 91202647

Watanabe M Kawamura S Nakada T Ishii N Hirano K Numasawa K Imamura A Left preureteral vena cava (retrocaval or circumcaval ureter) associated with partial situs inversus.

In: J Urol (1991 May) 145(5):1047-8

ISSN: 0022-5347

We present a rare case of left preureteral vena cava associated with partial situs inversus. A 68-year-old woman was referred to our clinic for further study of left hydronephrosis on computerized tomography. The abdominal viscera were in mirror image and the heart was levocardia. The middle portion of the left ureter was dorsal to the left inferior vena cava. The left ureter was reanastomosed ventral to the inferior vena cava. To our knowledge, coexistence of preureteral vena cava and partial situs inversus has not been reported previously in the literature.

Institutional address: Department of Urology Yamagata University School of Medicine Shinjo Prefectural Hospital Japan.

(REFERENCE 37 OF 41) 87141408

Matwijiw I Thliveris JA Faiman C Aplasia of nasal cilia with situs inversus, azoospermia and normal sperm flagella: a unique variant of the immotile cilia syndrome.

In: J Urol (1987 Mar) 137(3):522-4

ISSN: 0022-5347

A 23-year-old man with situs inversus and bronchiectasis was investigated because of azoospermia. Serum levels of testosterone and pituitary gonadotropins were normal. Scrotal exploration revealed no abnormalities of the testes, epididymides or vasa deferentia. Electron microscopy of a testicular biopsy specimen revealed a normal sperm flagellum structure, including the presence of dynein arms but 2 separate biopsies of the nasal mucosa revealed complete ciliary aplasia. This case, which bears a striking resemblance to Young's syndrome (bronchiectasis and azoospermia), represents a unique variant of the immotile cilia syndrome.

*****RADIOLOGIC CLINICS OF NORTH AMERICA*****

(REFERENCE 38 OF 41) 90068916

Winer-Muram HT Tonkin IL The spectrum of heterotaxic syndromes.

In: Radiol Clin North Am (1989 Nov) 27(6):1147-70

ISSN: 0033-8389

The heterotaxic syndromes may manifest as complex disorders with multiple anomalies, which have been described in the polysplenia/asplenia syndromes, or as a single isolated anomaly. Clinically insignificant anomalies may be incidentally discovered and should be recognized. Early diagnosis in the prenatal and neonatal periods allows for early intervention and correction, particularly of cardiac anomalies. Chest radiography, sonography, CT, radionuclide imaging, and MR imaging are useful diagnostic tools in evaluating these patients.

Institutional address: University of Tennessee Memphis.

*****RADIOLOGY*****

(REFERENCE 39 OF 41) 77103546

Markowitz RI Shashikumar VL Capitanio MA Volvulus of the colon in a child with congenital asplenia (Ivemark's syndrome).

In: Radiology (1977 Feb) 122(2):442

ISSN: 0033-8419

Volvulus of the colon occurred in a child with congenital asplenia (Ivemark's syndrome). This type of volvulus does not appear to have been reported previously and was found to be due to an underlying abnormality of mesenteric attachment.

*****SOUTHERN MEDICAL JOURNAL*****

(REFERENCE 40 OF 41) 79249043

Carmichael KA Gayle WE Jr Situs inversus and appendicitis.

In: South Med J (1979 Sep) 72(9):1147-50

ISSN: 0038-4348

Two cases of left lower quadrant appendicitis associated with situs inversus totalis are presented. Historic, genetic, and embryologic aspects of situs inversus, as well as recent theories regarding etiology are discussed. The clinical recognition of appendicitis in situs inversus is often difficult because of the unusual location of the appendix and abnormal pain localization. The differentiation of situs inversus totalis and abnormal rotation of the abdominal viscera (situs indeterminus) is important surgically because fixation of the midgut loop may be indicated in patients with situs indeterminus.

*****SURGERY*****

(REFERENCE 41 OF 41) 93362059

Klintmalm GB Bell MS Husberg BS Holman MJ Goldstein RM Ramsay MA Polter DE Liver transplant in complete situs inversus: a case report.

In: Surgery (1993 Jul) 114(1):102-6

ISSN: 0039-6060

BACKGROUND. Several technical solutions have been proposed for patients with situs inversus (SI) needing liver transplantation. This report describes the hepatic replacement in a patient with complete SI with dextrocardia. In the only other reported patient with complete SI the donor right lobe was placed over the vertebral column and the stomach to align the donor cava with that of the recipient. METHODS. A 45-year-old woman with complete SI, suffering from Laennec's cirrhosis with frequent upper gastrointestinal hemorrhages, underwent transplantation in June 1991. The recipient weighed 48.2 kilograms and was 155 centimeters tall. The donor weighed 77.3 kilograms and was 188 centimeters tall. The weight of the native liver was 1934 grams, and the donor liver weighed 1595 grams. RESULTS. At hepatectomy of the native liver an intact vena cava was left behind. Donor liver was rotated 90 degrees to the left, making the donor left lobe point into the left iliac fossa and the donor right lobe fall into the recipient hepatic fossa. Donor infrahepatic vena cava was sewn end-to-side to the recipient vena cava. Suprahepatic vena cava was oversewn. Donor and recipient hila were well aligned, allowing a standard arterial reconstruction and a choledocholedochostomy. Patient's recovery was uneventful with no problems during the following 6 months. CONCLUSIONS. The proposed technique for a patient with complete SI and dextrocardia offers several advantages: no need to downsize the donor in comparison with the recipient; no need for cutdown of the liver; no risk of kinking of the venous outflow; hepatic hila are aligned; and it allows for a standard arterial and biliary reconstruction. We recommend this technique as a procedure of choice for patients with SI and an intact vena cava.

Institutional address: Transplantation Services Baylor University Medical Center Dallas Texas 75246.