History

Cor triatriatum is essentially a form of left atrial inflow obstruction and presents with signs and symptoms of pulmonary venous obstruction.

Most patients present during infancy with a restrictive opening in the membrane. These infants usually present with evidence of low cardiac output, including pallor, diminished peripheral pulses, and tachypnea.

Feeding difficulties, poor weight gain, and respiratory distress are common.

Presentation later in life is less classic; however, when the patient becomes symptomatic, evidence of pulmonary venous obstruction predominates. In these patients, the membrane may become calcified with its orifice becoming smaller or the patient may develop mitral valve insufficiency. These patients may also present with arrhythmias secondary to an enlarged, hypertensive atrium.

Physical Examination

Despite the intracardiac defect, no characteristic murmur or pathognomonic physical characteristics are present in patients with cor triatriatum. This often leads to incorrect diagnosis of primary pulmonary vascular or parenchymal disease.

Signs of pulmonary venous obstruction and pulmonary hypertension are present. A right ventricular lift and accentuation of the pulmonary second sound are frequent and may be accompanied by an early diastolic murmur of pulmonary insufficiency. Rales may be present in the lung bases.

A soft, continuous murmur may be present due flow across the membrane. A murmur at the left sternal border is heard in patients with an atrial septal defect and a left-to-right shunt. A diastolic rumble of mitral stenosis at the apex is generally not heard with cor triatriatum.

Low cardiac output manifests as pallor, tachypnea, and diminished peripheral pulses.

Children are typically small, suffering from poor weight gain.

Patients presenting later in life may be dyspneic with a history of frequent pulmonary infections. They may have signs of right-sided heart failure, including distended peripheral veins and hepatomegaly. Signs and symptoms of pulmonary hypertension may be severe.

Differential Diagnoses

Humpl T, Reineker K, Manlhiot C, Dipchand AI, Coles JG, McCrindle BW. Cor triatriatum sinistrum in childhood. A single institution's experience. Can J Cardiol. 2010 Aug-Sep. 26(7):371-6. [QxMD MEDLINE Link]. [Full Text].
Fesslova V, Saracino A, Nuri H, Pome G. Cor triatriatum dexter: unusual features in utero and after birth. Interact Cardiovasc Thorac Surg. 2012 Mar. 14(3):330-2. [QxMD MEDLINE Link]. [Full Text].
Lasa JJ, Westover T, Khandelwal M, Cohen MS. Cor triatriatum dexter and right ventricular hypoplasia in a fetus. J Ultrasound Med. 2011 Dec. 30(12):1744-7. [QxMD MEDLINE Link].
Aliyu I, Ibrahim ZF. Cor-triatriatum dexter with associated cyanosis in a 3-month-old girl. J Cardiovasc Echogr. 2018 Apr-Jun. 28 (2):143-5. [QxMD MEDLINE Link]. [Full Text].
Vaideeswar P, Tullu MS, Sathe PA, Nanavati R. Atresia of the common pulmonary vein--a rare congenital anomaly. Congenit Heart Dis. 2008 Nov-Dec. 3(6):431-4. [QxMD MEDLINE Link].
Ito M, Kikuchi S, Hachiro Y, Abe T. Congenital pulmonary vein stenosis associated with cor triatriatum. Ann Thorac Surg. 2001 Feb. 71(2):722-3. [QxMD MEDLINE Link].
Bladt O, Vanhoenacker R. Cor triatriatum. JBR-BTR. 2008 Mar-Apr. 91(2):62. [QxMD MEDLINE Link].
Isik O, Akyuz M, Ayık MF, Levent E, Atay Y. Cor triatriatum sinister: a case series. Turk Kardiyol Dern Ars. 2016 Jan. 44 (1):20-3. [QxMD MEDLINE Link].
Vayngortin T, Rosenfeld H, Mansour K. A rare cause of shock in an infant: cor triatriatum. Pediatr Emerg Care. 2019 Sep. 35 (9):e172-3. [QxMD MEDLINE Link].
Moscoso B, Congiu S, Mayol J, Walter C, Caffarena JM. A rare case of cardiogenic shock: cor triatriatum with anomalous pulmonary venous return. World J Pediatr Congenit Heart Surg. 2019 Sep. 10 (5):651-3. [QxMD MEDLINE Link].
Burger C. A rare case of cor triatriatum leading to respiratory failure and shock in an 8-month-old boy. Pediatr Emerg Care. 2019 Jan. 35 (1):e6-e8. [QxMD MEDLINE Link].
Yozgat CY, Cakir E, Yazan H, Temur HO, Yakut K, Yozgat Y. A rare cause of pulmonary hypertension in a 4-year-old toddler: association of cor triatriatum sinister and pulmonary arteriovenous malformation. Case Rep Pediatr. 2020. 2020:8825215. [QxMD MEDLINE Link].
Spengos K, Gialafos E, Vassilopoulou S. Ischemic stroke as an uncommon complication of Cor triatriatum. J Stroke Cerebrovasc Dis. 2008 Nov-Dec. 17(6):436-8. [QxMD MEDLINE Link].
Leon RL, Zaban NB, Schamberger MS, Ho CY, Mietzsch U. Cyanosis and stroke due to functional cor triatriatum dexter in a neonate. Neonatology. 2018. 113 (3):231-4. [QxMD MEDLINE Link].
Yamada T, Tabereaux PB, McElderry HT, Doppalapudi H, Kay GN. Transseptal catheterization in the catheter ablation of atrial fibrillation in a patient with cor triatriatum sinister. J Interv Card Electrophysiol. 2009 Jun. 25(1):79-82. [QxMD MEDLINE Link].
Sormani P, Roghi A, Cereda A, et al. Partial anomalous pulmonary venous return as rare cause of right ventricular dilation: a retrospective analysis. Congenit Heart Dis. 2016 Jul. 11 (4):365-8. [QxMD MEDLINE Link]. [Full Text].
Kumar PA, Martinelli SM, Kyle RW, Arora H. Echocardiographic discovery of doubled interatrial septum: an incidental finding. Semin Cardiothorac Vasc Anesth. 2016 Sep. 20 (3):237-9. [QxMD MEDLINE Link]. [Full Text].
Modi KA, Annamali S, Ernest K, Pratep CR. Diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal and contrast echocardiography, and a review of literature. Echocardiography. 2006 Jul. 23(6):506-9. [QxMD MEDLINE Link].
Saremi F, Gurudevan SV, Narula J, Abolhoda A. Multidetector computed tomography (MDCT) in diagnosis of "cor triatriatum sinister". J Cardiovasc Comput Tomogr. 2007 Dec. 1(3):172-4. [QxMD MEDLINE Link].
Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. 2009 May. 192(5):1272-85. [QxMD MEDLINE Link].
Su CS, Tsai IC, Lin WW, Lee T, Ting CT, Liang KW. Usefulness of multidetector-row computed tomography in evaluating adult cor triatriatum. Tex Heart Inst J. 2008. 35(3):349-51. [QxMD MEDLINE Link].
Locca D, Hughes M, Mohiaddin R. Cardiovascular magnetic resonance diagnosis of a previously unreported association: Cor triatriatum with right partial anomalous pulmonary venous return to the azygos vein. Int J Cardiol. 2008 Jul 17. [QxMD MEDLINE Link].
Elagha AA, Fuisz AR, Weissman G. Cardiac magnetic resonance imaging can clearly depict the morphology and determine the significance of cor triatriatum. Circulation. 2012 Sep 18. 126(12):1511-3. [QxMD MEDLINE Link].
Abdel Razek AAK, Al-Marsafawy H, Elmansy M, El-Latif MA, Sobh D. Computed tomography angiography and magnetic resonance angiography of congenital anomalies of pulmonary veins. J Comput Assist Tomogr. 2019 May/Jun. 43 (3):399-405. [QxMD MEDLINE Link].
Kir M, Ugurlu B, Saylam GS, Karadas U. Surgical treatment of cor triatriatum, ventricular septal defect in an infant with a giant omphalocele. J Pediatr Surg. 2011 Jul. 46(7):E23-5. [QxMD MEDLINE Link].
Bartel T, Muller S, Erbel R. Dynamic three-dimensional echocardiography using parallel slicing: a promising diagnostic procedure in adults with congenital heart disease. Cardiology. 1998. 89(2):140-7. [QxMD MEDLINE Link].
Citro R, Bossone E, Provenza G, Patella MM, Gregorio G. Isolated left cor triatriatum: a rare cause of effort dyspnoea in the adult. J Cardiovasc Med (Hagerstown). 2008 Sep. 9(9):926-8. [QxMD MEDLINE Link].
Gharagozloo F, Bulkley BH, Hutchins GM. A proposed pathogenesis of cor triatriatum: impingement of the left superior vena cava on the developing left atrium. Am Heart J. 1977 Nov. 94(5):618-26. [QxMD MEDLINE Link].
Jeiger W, Gibbons JE, Wigglesworth FW. Cor triatriatum: Clinical, hemodynamic and pathologic studies: Surgical correction in early life. Pediatrics. 1963. 31:255-64.
Kerkar P, Vora A, Kulkarni H, et al. Percutaneous balloon dilatation of cor triatriatum sinister. Am Heart J. 1996 Oct. 132(4):888-91. [QxMD MEDLINE Link].
Kirklin JW, Barratt-Boyes BG. Cardiac Surgery. 2nd ed. Churchill Livingstone; 1993. 675-81.
Marini D, Ou P. Cor triatriatum in a newborn. Pediatr Radiol. 2009 Mar 10. [QxMD MEDLINE Link].
McLean MK, Kung GC, Polimenakos A, Wells WJ, Reemtsen BL. Cor triatriatum associated with ASD and common atrium in 7-month-old with tachypnea and failure to thrive. Ann Thorac Surg. 2008 Dec. 86(6):1999. [QxMD MEDLINE Link].
Oglietti J, Cooley DA, Izquierdo JP, et al. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg. 1983 Apr. 35(4):415-20. [QxMD MEDLINE Link].
[Guideline] Paridon SM, Alpert BS, Boas SR, et al. Clinical stress testing in the pediatric age group: a statement from the American Heart Association Council on Cardiovascular Disease in the Young, Committee on Atherosclerosis, Hypertension, and Obesity in Youth. Circulation. 2006 Apr 18. 113(15):1905-20. [QxMD MEDLINE Link].
Richardson JV, Doty DB, Siewers RD, Zuberbuhler JR. Cor triatriatum (subdivided left atrium). J Thorac Cardiovasc Surg. 1981 Feb. 81(2):232-8. [QxMD MEDLINE Link].
Rodefeld MD, Brown JW, Heimansohn DA, et al. Cor triatriatum: clinical presentation and surgical results in 12 patients. Ann Thorac Surg. 1990 Oct. 50(4):562-8. [QxMD MEDLINE Link].
Salomone G, Tiraboschi R, Bianchi T, et al. Cor triatriatum. Clinical presentation and operative results. J Thorac Cardiovasc Surg. 1991 Jun. 101(6):1088-92. [QxMD MEDLINE Link].
Spencer FC, Sabiston DC. Surgery of the Chest. 6th ed. WB Saunders; 1995. 1420-4.
Sritippayawan S, Margetis MF, MacLaughlin EF, et al. Cor triatriatum: a cause of hemoptysis. Pediatr Pulmonol. 2002 Nov. 34(5):405-8. [QxMD MEDLINE Link].
Su CS, Tsai IC, Lin WW, Lee T, Ting CT, Liang KW. Usefulness of multidetector-row computed tomography in evaluating adult cor triatriatum. Tex Heart Inst J. 2008. 35(3):349-51. [QxMD MEDLINE Link].
Tantibhedhyangkul W, Godoy I, Karp R, Lang RM. Cor triatriatum in a 70-year-old woman: role of transesophageal echocardiography and dynamic three-dimensional echocardiography in diagnostic assessment. J Am Soc Echocardiogr. 1998 Aug. 11(8):837-40. [QxMD MEDLINE Link].
Rozema TK, Arruda J, Snyder CS. Cor triatriatum: a tale of two membranes. CASE (Phila). 2019 Feb. 3 (1):25-7. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Pediatric Cor Triatriatum. Long-axis parasternal view demonstrating a left atrial membrane separating pulmonary vein inflow from left ventricular (mitral valve) inflow. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Long-axis parasternal view depicting a two-dimensional image of cor triatriatum sinister membrane and a color Doppler image of pulmonary venous flow through the orifice of the membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Short-axis parasternal view depicting right and left pulmonary vein flow proximal to the cor triatriatum left atrial membrane and left atrial appendage orifice distal to the cor triatriatum left atrial membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Subxiphoid coronal image of the posterior left atrial chamber that receives pulmonary venous flow separated from the rest of the left atrium by the cor triatriatum membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Apical five-chamber view demonstrating a 4-5 mm left atrial membrane orifice with mild pulmonary venous inflow restriction. Note the presence of an associated perimembranous ventricular septal defect (VSD) with tricuspid septal aneurysmal tissue. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. This sonogram shows a mean Doppler gradient of 7-8 mmHg across the left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

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Author

M Silvana Horenstein, MD Assistant Professor, Department of Pediatrics, University of Texas Medical School at Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Maria Victoria T Tantengco, MD Associate Professor of Pediatrics, Division of Cardiology, Department of Pediatrics, University of Massachusetts Medical School; Medical Director, Echocardiography Laboratory, Child Heart Associates, LLC

Maria Victoria T Tantengco, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Massachusetts Medical Society, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous coauthors Manuel Caceres, MD; James Jaggers, MD; and Jeff L Myers, MD, PhD, to the writing and development of this article.

Pediatric Cor Triatriatum Clinical Presentation

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