Pediatric Cor Triatriatum

No Results

No Results

processing….

Cor triatriatum is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the atrium in two. In its most common form, cor triatriatum sinister, the left atrium is divided into an upper chamber that receives the pulmonary veins and a lower chamber that is related to the left atrial appendage and the mitral valve orifice. [1]

The location of the atrial appendage is a key landmark in this congenital malformation. It differentiates cor triatriatum from a physiologically similar condition, supravalvular mitral stenosis. In cor triatriatum the left atrial appendage is invariably associated with the lower chamber, which is below the membrane.

Rarely, a fibromuscular membrane that is a remnant of the right valve of the sinus venosus divides the right atrium; this finding, so-called cor triatriatum dexter, is usually asymptomatic and is mostly reported as an incidental finding. [2] However, fetal right ventricular hypoplasia has been described associated with this anomaly and has been hypothesized to result from abnormal fetal circulation leading to growth restriction of the fetal right ventricle. [3] Owing to its clinical relevance, the remainder of this chapter focuses on cor triatriatum sinister.

Cor triatriatum represents 0.1% of all congenital cardiac malformations and may be associated with other cardiac defects in as many as 50% of cases. Examples of associated cardiac defects include atrial septal defect, persistent left superior vena cava with an unroofed coronary sinus, partial anomalous pulmonary venous connection, ventricular septal defect, and more complex cardiac lesions, such as tetralogy of Fallot, atrioventricular canal, and double outlet right ventricle. Associated bicuspid pulmonary valve, aortic valve atresia, and heterotaxy have also been described. [4]

In classic cor triatriatum, the pulmonary venous chamber receives all pulmonary veins and it drains into the left atrium through a variable-sized orifice. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chambers. If the communicating orifice is small, the patient presents critically ill and may succumb at a young age (usually during infancy) to congestive heart failure and pulmonary edema. If the connection is larger, patients may present in childhood or young adulthood with a clinical picture similar to that of mitral stenosis. Cor triatriatum may also be an incidental finding when it is nonobstructive.

Other anatomical variants of cor triatriatum include a pulmonary venous chamber that receives part of the pulmonary veins and communicates with the left atrium, and a pulmonary venous chamber receiving all or part of the pulmonary veins that does not connect with the left atrium.

Congenital pulmonary vein stenosis is a very rare association with cor triatriatum. [5] Variable types of subtotal cor triatriatum may be noted, with only the right or left pulmonary veins draining into the upper chamber. [6]

Initially, the fetal lungs and pulmonary veins are connected to the systemic venous circulation. Subsequently, a dorsal outgrowth from the common atria, also referred as common pulmonary vein, evaginates and joins the pulmonary veins, whereas the connection to the systemic circulation disappears. As the fetal heart grows, the common pulmonary vein is completely absorbed. Failure of this dorsal outgrowth to join the pulmonary veins results in total anomalous pulmonary venous drainage (TAPVD). An abnormal connection between the common pulmonary vein and the atria results in any of the variants of cor triatriatum.

The critical anatomic feature of cor triatriatum is a diaphragm that divides the left atrium into 2 chambers (see the videos below).

It consists of fibromuscular tissue, and the proximal chamber that is created represents a vestigial common pulmonary vein. The pulmonary veins thus drain into the proximal chamber.

Outcome depends on the size of the communication to the distal chamber, which communicates with the mitral valve. If present, a patent fossa ovalis or secundum atrial septal defect permits decompression of the proximal chamber into the right atrium.

The presentation of cor triatriatum is one of decreased cardiac output and pulmonary venous hypertension. If a connection between the common pulmonary venous chamber and the right atrium is present, pulmonary overcirculation may result in significant right ventricular enlargement.

United States

This is a very rare malformation. Incidence is approximately 0.1-0.4% of all infants with congenital cardiac disease.

Approximately 75% of patients die in infancy (generally from pulmonary hypertension) if the defect is unrepaired. If the communication between the proximal and distal chambers is not restrictive or if an atrial septal defect allows decompression of the hypertensive left atrium, the prognosis is significantly improved.

The only treatment is surgical correction. Most postoperative deaths occur in the first 30 days. The early mortality rate in each large series was consistently 15-20%. Early deaths had a higher rate of associated severe cardiac anomalies.

Long-term results are excellent, with long term survival of 80-90% in patients surviving surgery. Survivors have excellent functional results without residual sequelae and a life expectancy that approaches that of the general population. This is particularly true when repair is performed in infancy.

Complications

If the condition is unrecognized and untreated, problems include pulmonary edema, right heart failure, and death.

Late complications include recurrence of the obstructive membrane with recurrence of symptoms. [5] Similar symptoms may occur later on in life if the membrane is incompletely excised.

Ischemic stroke as a late complication from undiagnosed cor triatriatum (sinister) in an adult has been described. [7]

No race predilection is reported.

A slight male predilection is observed, with a male-to-female ratio of 1.4:1.

Diagnosis is primarily made in infancy. Later presentation does occur but is usually in childhood or early adulthood; in this patient population, 85% are younger than 40 years. Rarely, patients presenting in their eighth or ninth decade of life have been reported.

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. [Medline]. [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. [Medline]. [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. [Medline].

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. [Medline].

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. [Medline].

Bladt O, Vanhoenacker R. Cor triatriatum. JBR-BTR. 2008 Mar-Apr. 91(2):62. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. 2009 May. 192(5):1272-85. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

Oglietti J, Cooley DA, Izquierdo JP, et al. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg. 1983 Apr. 35(4):415-20. [Medline].

[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. [Medline].

Richardson JV, Doty DB, Siewers RD, Zuberbuhler JR. Cor triatriatum (subdivided left atrium). J Thorac Cardiovasc Surg. 1981 Feb. 81(2):232-8. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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. [Medline].

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.

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.

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.

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.

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.

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

Research & References of Pediatric Cor Triatriatum|A&C Accounting And Tax Services
Source