Research & Literature

Ivemark syndrome-a rare entity with specific anatomical features.

Hrusca A, Rachisan AL, Lucian B, et al.

Revista medica de Chile 2015; (143(3)):383-6.

Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy.

Loomba R, Shah PH, Anderson RH

Cureus 2015; (7(5)):e269 doi:10.7759/cureus.269.

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism.

Loomba RS, Willes RJ, Kovach JR, Anderson RH

Congenital heart disease 2016; (11(1)):7-18 doi:10.1111/chd.12288.

Surgical Treatment of Dextroversion, Isolated Persistent Left Superior Vena Cava Draining Into the Left Atrium.

Zhong YL, Long XM, Jiang LY, et al.

Journal of cardiac surgery 2015; (30(10)):767-70 doi:10.1111/jocs.12611.

A review of guidance on immunization in persons with defective or deficient splenic function.

Kuchar E, Miśkiewicz K, Karlikowska M

British journal of haematology 2015; (171(5)):683-94 doi:10.1111/bjh.13660.

Observation versus prophylactic Ladd procedure for asymptomatic intestinal rotational abnormalities in heterotaxy syndrome: A systematic review.

Landisch R, Abdel-Hafeez AH, Massoumi R, et al.

Journal of pediatric surgery 2015; (50(11)):1971-4.

Right Atrial Flap Repair for Left Superior Vena Cava Draining into Left Atrium.

Erek E, Aydin S, Suzan D

The Thoracic and cardiovascular surgeon 2016; (64(1)):59-61 doi:10.1055/s-0035-1564931.

Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection.

Chiu SN, Shao PL, Wang JK, et al.

Pediatric research 2016; (79(2)):271-7 doi:10.1038/pr.2015.221.

Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome.

Chen W, Ma L, Cui H, et al.

Cardiology 2016; (133(3)):141-6 doi:10.1159/000440947.

Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome.

Mishra S

Indian journal of pediatrics 2015; (82(12)):1135-46 doi:10.1007/s12098-015-1925-x.

Overwhelming Postsplenectomy Infection: A Prospective Multicenter Cohort Study.

Theilacker C, Ludewig K, Serr A, et al.

Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 2016; (62(7)):871-878 doi:10.1093/cid/civ1195.

Evaluating a management strategy for malrotation in heterotaxy patients.

Abbas PI, Dickerson HA, Wesson DE

Journal of pediatric surgery 2016; (51(5)):859-62.

Medical complications following splenectomy.

Buzelé R, Barbier L, Sauvanet A, Fantin B

Journal of visceral surgery 2016; (153(4)):277-86.

Intestinal Rotation Anomalies.

Pelayo JC, Lo A

Pediatric annals 2016; (45(7)):e247-50 doi:10.3928/00904481-20160602-01.

Intestinal malrotation and midgut volvulus.

Hamidi H, Obaidy Y, Maroof S

Radiology case reports 2016; (11(3)):271-4 doi:10.1016/j.radcr.2016.05.012.

Intestinal malrotation in patients with situs anomaly: Implication of the relative positions of the superior mesenteric artery and vein.

Choi KS, Choi YH, Cheon JE, et al.

European journal of radiology 2016; (85(10)):1695-1700 doi:10.1016/j.ejrad.2016.07.013.

Primary Care of the Blunt Splenic Injured Adult.

Yorkgitis BK

The American journal of medicine 2017; (130(3)):365.e1-365.e5 doi:10.1016/j.amjmed.2016.10.010.

The impact of cardiac risk factors on short-term outcomes for children undergoing a Ladd procedure.

Putnam LR, Anderson KT, Tsao K, et al.

Journal of pediatric surgery 2017; (52(3)):390-394 doi:10.1016/j.jpedsurg.2016.09.064.

Spleen Australia guidelines for the prevention of sepsis in patients with asplenia and hyposplenism in Australia and New Zealand.

Kanhutu K, Jones P, Cheng AC, et al.

Internal medicine journal 2017; (47(8)):848-855 doi:10.1111/imj.13348.

Intestinal Malrotation and Volvulus in Neonates: Laparoscopy Versus Open Laparotomy.

Ferrero L, Ahmed YB, Philippe P, et al.

Journal of laparoendoscopic & advanced surgical techniques. Part A 2017; (27(3)):318-321 doi:10.1089/lap.2015.0544.

Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease.

Rankine-Mullings AE, Owusu-Ofori S

The Cochrane database of systematic reviews 2017; (10()):CD003427 doi:10.1002/14651858.CD003427.pub4.

Association between defective spleen function and primary eosinophilic gastrointestinal disorders.

Di Sabatino A, Aronico N, Giuffrida P, et al.

The journal of allergy and clinical immunology. In practice 2018; (6(3)):1056-1058.e1 doi:10.1016/j.jaip.2017.10.017.

Sporadic isolated congenital asplenia with fulminant pneumococcal meningitis: a case report and updated literature review.

Iijima S

BMC infectious diseases 2017; (17(1)):777 doi:10.1186/s12879-017-2896-5.

A Registry for Patients With Asplenia/Hyposplenism Reduces the Risk of Infections With Encapsulated Organisms.

Arnott A, Jones P, Franklin LJ, et al.

Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 2018; (67(4)):557-561 doi:10.1093/cid/ciy141.

Raised Anxiety Levels Among Outpatients Preparing to Undergo a Medical Imaging Procedure: Prevalence and Correlates.

Forshaw KL, Boyes AW, Carey ML, et al.

Journal of the American College of Radiology : JACR 2018; (15(4)):630-638 doi:10.1016/j.jacr.2017.12.030.

An adult presentation of midgut volvulus secondary to intestinal malrotation: A case report and literature review.

Butterworth WA, Butterworth JW

International journal of surgery case reports 2018; (50()):46-49 doi:10.1016/j.ijscr.2018.07.007.

A novel ZIC3 gene mutation identified in patients with heterotaxy and congenital heart disease.

Li S, Liu S, Chen W, et al.

Scientific reports 2018; (8(1)):12386 doi:10.1038/s41598-018-30204-3.

Complex congenital cardiac anomalies in the setting of right isomerism in a 31-month-old infant: a case report.

Lyimo FR, Pallangyo P, Majani N, et al.

Journal of medical case reports 2018; (12(1)):324 doi:10.1186/s13256-018-1835-4.

Successful Palliation via Kawashima Procedure of an Infant With Heterotaxy Syndrome and Left-Atrial Isomerism.

Lively-Endicott H, Lara DA

Ochsner journal 2018; (18(4)):406-412 doi:10.31486/toj.18.0042.

Pulmonary Hemodynamic Changes with Nitric Oxide or Oxygen in a Patient with Asplenia, Single Right Ventricle, and Total Anomalous Pulmonary Venous Connection after Fontan Procedure.

Oka H, Nakau K, Kajihama A, et al.

Case reports in cardiology 2018; (2018()):3736254 doi:10.1155/2018/3736254.

Low-risk factors for severe bacterial infection and acute chest syndrome in children with sickle cell disease.

Rincón-López EM, Navarro Gómez ML, Hernández-Sampelayo Matos T, et al.

Pediatric blood & cancer 2019; (66(6)):e27667 doi:10.1002/pbc.27667.

DNAH11 variants and its association with congenital heart disease and heterotaxy syndrome.

Liu S, Chen W, Zhan Y, et al.

Scientific reports 2019; (9(1)):6683 doi:10.1038/s41598-019-43109-6.

Characterisation of computed tomography angiography findings in paediatric patients with heterotaxy.

Ugas Charcape CF, Alpaca Rodriguez LR, Matos Rojas IA, et al.

Pediatric radiology 2019; (49(9)):1142-1151 doi:10.1007/s00247-019-04434-0.

Post-splenectomy sepsis: preventative strategies, challenges, and solutions.

Luu S, Spelman D, Woolley IJ

Infection and drug resistance 2019; (12()):2839-2851 doi:10.2147/IDR.S179902.

Pulmonary venous obstruction after extracardiac total cavopulmonary connection in right atrial isomerism.

Kugo Y, Iwai S, Ishimaru K, et al.

General thoracic and cardiovascular surgery 2020; (68(9)):969-974 doi:10.1007/s11748-020-01316-3.

Isolated Congenital Asplenia in an Asymptomatic Patient: A Very Rare Diagnosis.

Dinis-Ferreira S, Lemos C, Caldeira M, et al.

European journal of case reports in internal medicine 2020; (7(4)):001429 doi:10.12890/2020_001429.

[Benefit of Howell-Jolly bodies detection: finding of an acquired hyposplenism in a patient with Goodpasture syndrome].

Leroux T, Daliphard S, Etienne I, et al.

Annales de biologie clinique 2020; (78(2)):191-194 doi:10.1684/abc.2020.1544.

Making the diagnosis of midgut volvulus: Limited abdominal ultrasound has changed our clinical practice.

Wong K, Van Tassel D, Lee J, et al.

Journal of pediatric surgery 2020; (55(12)):2614-2617 doi:10.1016/j.jpedsurg.2020.04.012.

A novel DNAH11 variant segregating in a sibship with heterotaxy and implications for genetic counseling.

Namavarian A, Eid A, Goh ES, Thakur V

Molecular genetics & genomic medicine 2020; (8(9)):e1358 doi:10.1002/mgg3.1358.

Asplenia and Hyposplenism: An Underrecognized Immune Deficiency.

Squire JD, Sher M

Immunology and allergy clinics of North America 2020; (40(3)):471-483 doi:10.1016/j.iac.2020.03.006.

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

Okamoto T, Nakano T, Goda M, et al.

General thoracic and cardiovascular surgery 2021; (69(4)):646-653 doi:10.1007/s11748-020-01474-4.

Overwhelming Post-Splenectomy Infection Syndrome: Variability in Timing With Similar Presentation.

Gupta AK, Vazquez OA

Cureus 2020; (12(8)):e9914 doi:10.7759/cureus.9914.

Laparoscopic sleeve gastrectomy in polysplenia syndrome/left isomerism: A case report.

Jeragh F, Aljazzaf I, Al Khayyat H

International journal of surgery case reports 2020; (75()):488-491 doi:10.1016/j.ijscr.2020.09.079.

Unique foetal diagnosis of aorto-pulmonary collaterals in right atrial isomerism.

Mawad W, Dutil N, Thakur V

Cardiology in the young 2021; (31(2)):303-305 doi:10.1017/S1047951120003649.

Twin atrioventricular nodes, arrhythmias, and survival in pediatric and adult patients with heterotaxy syndrome.

Wu MH, Wang JK, Chiu SN, et al.

Heart rhythm 2021; (18(4)):605-612 doi:10.1016/j.hrthm.2020.12.012.

Renal replacement therapy with a cytokine-adsorbing hemofilter after neonatal open-heart surgery.

Shimada M, Hoashi T, Imai K, Ichikawa H

Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs 2021; (24(3)):398-401 doi:10.1007/s10047-020-01241-5.

The patient perspective in the era of personalized medicine: What about scanxiety?

Custers JAE, Davis L, Messiou C, et al.

Cancer medicine 2021; (10(9)):2943-2945 doi:10.1002/cam4.3889.

Heterotaxy Syndrome: Discordant Growth.

Yadav P, Ajmera P, Krishnamurthy S, Nandivada NB

Cureus 2021; (13(4)):e14766 doi:10.7759/cureus.14766.

Ultrasound for malrotation and volvulus - point.

Youssfi M, Goncalves LF

Pediatric radiology 2022; (52(4)):716-722 doi:10.1007/s00247-021-05154-0.

Emergency surgical treatment of total anomalous pulmonary venous connection.

Wu Y, Fan X, Chen L, et al.

Journal of cardiac surgery 2022; (37(1)):47-52 doi:10.1111/jocs.16079.

Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction.

Chemello L, Padalino M, Zanon C, et al.

Journal of cardiovascular development and disease 2021; (8(10)) doi:10.3390/jcdd8100117.

Heterotaxy syndrome: Prenatal diagnosis, concomitant malformations and outcomes.

Akalın M, Demirci O, Kumru P, Yücel İK

Prenatal diagnosis 2022; (42(4)):435-446 doi:10.1002/pd.6110.

Utility of Three-Dimensional Printed Model in Biventricular Repair of Complex Congenital Cardiac Defects: Case Report and Review of Literature.

Betancourt LG, Wong SH, Singh HR, et al.

Children (Basel, Switzerland) 2022; (9(2)) doi:10.3390/children9020184.

Diagnosis and management of intestinal rotational abnormalities with or without volvulus in the pediatric population.

Svetanoff WJ, Srivatsa S, Diefenbach K, Nwomeh BC

Seminars in pediatric surgery 2022; (31(1)):151141 doi:10.1016/j.sempedsurg.2022.151141.

Letter to the Editor: Cardiac surgery in patients with atrial isomerism: Long-term results and outcomes.

Ahmed S, Sohaib M

Journal of cardiac surgery 2022; (37(6)):1796 doi:10.1111/jocs.16484.

Genetic and Clinical Features of Heterotaxy in a Prenatal Cohort.

Yi T, Sun H, Fu Y, et al.

Frontiers in genetics 2022; (13()):818241 doi:10.3389/fgene.2022.818241.

Clinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome.

Wang CC, Wu MH, Wu ET, et al.

Pediatric pulmonology 2022; (57(9)):2074-2081 doi:10.1002/ppul.25981.

A novel nonsense PKD1L1 variant cause heterotaxy syndrome with congenital asplenia in a Han Chinese patient.

Gu H, Yuan ZZ, Xie XH, et al.

Journal of human genetics 2022; (67(10)):573-577 doi:10.1038/s10038-022-01053-w.

A Novel Technique of SMA Massage with Systemic Fibrinolytic Therapy in Ischemic Midgut Volvulus: As a Lifesaving Last Expedient.

Giriradder VB, Jadhav V, Anilkumar PL, Babu MN

Journal of Indian Association of Pediatric Surgeons 2023; (28(1)):25-28 doi:10.4103/jiaps.jiaps_69_22.

Cardiac surgical outcomes of patients with heterotaxy syndrome.

Desai MH, Ceneri NM, Dhari Z, et al.

JTCVS open 2023; (13()):292-306 doi:10.1016/j.xjon.2022.12.004.

Scanxiety Conversations on Twitter: Observational Study.

Bui KT, Li Z, Dhillon HM, et al.

JMIR cancer 2023; (9()):e43609 doi:10.2196/43609.

Care Pattern for Fontan-Associated Liver Disease by Academic Pediatric Hepatologists in Canada.

Kehar M, Jimenez-Rivera C

JPGN reports 2022; (3(3)):e207 doi:10.1097/PG9.0000000000000207.

Characterization of ultrasound and postnatal pathology in fetuses with heterotaxy syndrome.

Wu Q, Guo S, Huang B, et al.

Frontiers in cardiovascular medicine 2023; (10()):1195191 doi:10.3389/fcvm.2023.1195191.

Unravelling the complexity of heterotaxy syndrome.

Bilamge A, Vallabhaneni P, Didel S, Rajagopal R

BMJ case reports 2023; (16(10)) doi:10.1136/bcr-2023-256198.

Cardiac Laterality: Surgical Results of Right Atrial Isomerism.

Ortega-Zhindón DB, Pérez-Hernández N, Rodríguez-Pérez JM, et al.

Diseases (Basel, Switzerland) 2023; (11(4)) doi:10.3390/diseases11040170.

Notable Clinical Differences Between Neonatal and Post-Neonatal Intestinal Malrotation: A Multicenter Review in Southern Japan.

Kedoin C, Muto M, Nagano A, et al.

Journal of pediatric surgery 2024; (59(4)):566-570 doi:10.1016/j.jpedsurg.2023.11.020.

Scanxiety and quality of life around follow-up imaging in patients with unruptured intracranial aneurysms: a prospective cohort study.

Kamphuis MJ, van der Kamp LT, van Eijk RPA, et al.

European radiology 2024; (34(9)):6018-6025 doi:10.1007/s00330-024-10602-0.

Infectious purpura fulminans associated with pneumococcal septicaemia in a patient with unacknowledged functional asplenia.

Duus S, Jespersen S, Wejse C

BMJ case reports 2024; (17(3)) doi:10.1136/bcr-2022-251397.

Heterotaxy Syndrome With Right Isomerism and Interrupted Inferior Vena Cava: A Case Report and Literature Review.

Iskafi RA, Abugharbieh Y, Ahmad I, et al.

Cureus 2024; (16(3)):e55698 doi:10.7759/cureus.55698.

British Society for Haematology guidelines to improve the care of asplenic patients: Much work done, some remaining and a call for national registries.

Rivière E

British journal of haematology 2024; (204(5)):1573-1576 doi:10.1111/bjh.19379.

Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome.

Alemany VS, Crawford A, Gauvreau K, et al.

JTCVS open 2024; (18()):167-179 doi:10.1016/j.xjon.2024.02.011.

Updated Recommendations on the Prevention and Treatment of Infections in Children With Asplenia/Hyposplenism.

Guri A, Ben-Ami T

Journal of pediatric hematology/oncology 2024; (46(5)):225-232 doi:10.1097/MPH.0000000000002855.

Ultrasound and computed tomography angiography diagnosis of fetal right atrial isomerism with cardiac total anomalous pulmonary venous connection and intestinal malrotation: a case report and literature review.

Gao J, Ou H, Xing H, et al.

Pediatric radiology 2024; (54(8)):1399-1404 doi:10.1007/s00247-024-05938-0.

A Rare Case of Delayed Presentation of Acute Midgut Volvulus in an Adolescent.

Nichat P, Gandhi AS, Rathod C, et al.

Cureus 2024; (16(6)):e62256 doi:10.7759/cureus.62256.

Effect of information delivery techniques in reducing pre-procedural anxiety in computed tomography.

Ahmed HS, Gupta D, Aluru DR, et al.

Current problems in diagnostic radiology 2024; (53(6)):723-727 doi:10.1067/j.cpradiol.2024.07.015.

A case report of a newborn who underwent ultra-high enterostomy and intestinal fluid return after necrosis of intestinal volvulus.

Cui L, Zhang X, Liu Y, Chen Y

Translational pediatrics 2024; (13(11)):2067-2076 doi:10.21037/tp-24-214.

Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia.

MacDonald B, Vetten Z, Ramsay J, et al.

International journal of cardiology. Congenital heart disease 2023; (14()):100472 doi:10.1016/j.ijcchd.2023.100472.

Splenic red pulp macrophages eliminate the liver-resistant Streptococcus pneumoniae from the blood circulation of mice.

An H, Huang Y, Zhao Z, et al.

Science advances 2025; (11(11)):eadq6399 doi:10.1126/sciadv.adq6399.

Improved Surgical Management of Complex Neonates With Heterotaxy Syndrome.

Mills AC, Dawson AE, Scott MC, et al.

World journal for pediatric & congenital heart surgery 2026; (17(1)):28-35 doi:10.1177/21501351251345791.

Genetic aspects of congenital heart disease in heterotaxy syndrome.

Ahmad Rafie NN, Yubbu P, Aissvarya S, et al.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology 2025; (44(10)):633-648 doi:10.1016/j.repc.2025.05.007.

Clinical relevance of the compartments and lymphocyte subsets in the human spleen.

Pabst R

Cell and tissue research 2025; (402(1)):109-119 doi:10.1007/s00441-025-04001-0.

The complex and hazardous course for heterotaxy-associated congenital heart disease.

Olds A, Meza JM, Rajeev N, et al.

JTCVS open 2025; (26()):196-206 doi:10.1016/j.xjon.2025.06.018.

Infant malrotation with midgut volvulus: A retrospective review of clinical presentation and delays in care at a Canadian tertiary paediatric centre.

Filion L, Beaunoyer M, Miron MC, et al.

Paediatrics & child health 2025; (30(6)):453-458 doi:10.1093/pch/pxaf042.

Multisystem Phenotypic Spectrum in Pediatric Heterotaxy Syndrome: A Case Series.

Mukherjee TG, Thurmann KE, Richardson RR

Cureus 2025; (17(9)):e91434 doi:10.7759/cureus.91434.

Specific cardiovascular morphology on computed tomography angiography in patients with right isomerism.

Tomita Y, Muneuchi J, Sugitani Y, et al.

Pediatrics international : official journal of the Japan Pediatric Society 2025; (67(1)):e70234 doi:10.1111/ped.70234.

Distinct Defects in Marginal Zone B Cells and Filtration Function Characterize Hyposplenism in Persons With HIV-1 on Prolonged ART.

Debiesse S, Bedin AS, Pisoni A, et al.

Open forum infectious diseases 2025; (12(11)):ofaf644 doi:10.1093/ofid/ofaf644.

The posterior tunnel Fontan: A different approach to lateral tunnel Fontan.

Aydin MI, O'Hara R, Bishnoi A, d'Udekem Y

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2025; doi:10.1093/ejcts/ezaf408.

From multiple spleens to absence: Insights from two cases of heterotaxy syndromes.

Paudel S, Banjade RR, Sharma NK, et al.

Radiology case reports 2026; (21(3)):1217-1223 doi:10.1016/j.radcr.2025.11.066.

Long-Term Outcomes and Risk Factors for Mortality After Single Ventricle Palliation for Asplenia Syndrome.

Kobayashi Y, Sano S, Tokuda Y, et al.

Journal of the American Heart Association 2026; (15(3)):e044756 doi:10.1161/JAHA.125.044756.