You are in: eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology Cor TriatriatumArticle Last Updated: Jul 6, 2006AUTHOR AND EDITOR INFORMATIONAuthor: Manuel Caceres, MD, Staff Physician, Department of Surgery, Division of Cardiothoracic Surgery, University of Tennessee Health Sciences Center Manuel Caceres is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, Society of Thoracic Surgeons, and Southern Medical Association Coauthor(s): James Jaggers, MD, Chief of Pediatric Cardiac Surgery, Professor, Department of Surgery, Division of Cardiovascular and Thoracic Surgery, Duke University Medical Center; Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School Editors: Juan Carlos Alejos, MD, Associate Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Ameeta Martin, MD, Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, University of Nebraska College of Medicine; Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Department of Pediatrics, Baylor College of Medicine Author and Editor Disclosure Synonyms and related keywords: cor triatriatum, cardiac anomaly, subdivided left atrium, accessory atrium, congenital heart disease, congenital cardiac anomaly INTRODUCTIONBackgroundCor triatriatum is a rare congenital cardiac anomaly, representing 0.1% of all congenital cardiac malformations, without any known associated genetic abnormalities. It has no gender predilection and is associated with other cardiac defects in up to 50% of cases. The term cor triatriatum was first used by Borst in 1905, and the first surgical repair was performed by Vineberg in 1956. In its most common form, cor triatriatum sinister, the left atrium is divided into a proximal and distal chamber. Both chambers are separated by a diaphragm with 1 or more restrictive ostia, with the pulmonary veins draining into the proximal chamber. Variable types of subtotal cor triatriatum also exist, with only the right or left pulmonary veins draining into the proximal chamber. The location of the atrial appendage is a key landmark in this congenital malformation. It differentiates cor triatriatum from a similar condition, supravalvular mitral stenosis. In cor triatriatum, the left atrial appendage is invariably found in the same chamber with the mitral valve ring, distal to the dividing atrial diaphragm. An embryologically unrelated membrane may rarely divide the right atrium; this finding, so-called cor triatriatum dexter, is usually asymptomatic and is mostly reported as an incidental finding. The natural history of this defect is dependent on the size of the ostia. If small, the infants are critically ill and succumb at a young age. If the connection is larger, patients present in childhood or young adulthood with a clinical picture similar to mitral stenosis. The latest reported presentation in life corresponds to a 70-year-old woman with an unrecognized nonobstructive cor triatriatum due to multiple fenestrations in the left atrial membrane. PathophysiologyInitially, 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, while 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 Image 1). 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. FrequencyUnited StatesThis is a very rare malformation. Incidence is probably 0.1-0.4% of all infants with congenital cardiac disease. In a combined series from the Mayo Clinic (1955-1967) and University of Alabama at Birmingham (1967-1991), only 10 patients underwent surgical repair. The Texas Heart Institute repaired defects in 25 patients over a 21-year period. Doty conducted a series at 2 institutions over a 23-year period that involved 21 patients. Mortality/Morbidity
SexThere appears to be a slight male predilection with a male-to-female ratio of 1.4:1. Age
CLINICALHistory
Physical
Causes
DIFFERENTIALSMitral Stenosis, Supravalvular Ring Partial Anomalous Pulmonary Venous Connection Pulmonary Hypertension, Idiopathic Pulmonary Hypertension, Persistent-Newborn Pulmonary Hypoplasia Total Anomalous Pulmonary Venous Connection
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| Drug Name | Digoxin (Lanoxin) |
|---|---|
| Description | Exerts its inotropic action by increasing the amount of intracellular calcium available during excitation-contraction coupling. It is one of numerous inotropic agents that can be used in infants with congenital cardiac defects. Other agents, such as dopamine (described below), are more appropriate for acute management of heart failure in ICU setting. |
| Adult Dose | Total digitalizing dose (TDD): 0.75-1.5 mg PO; 0.5-1 mg IV/IM Administer 50% of TDD initially; remaining 2 doses at 25% TDD q6-12h Maintenance dose: 0.125-0.5 mg/d PO; 0.1-0.4 mg/d IV/IM |
| Pediatric Dose | TDD PO: Preterm infant: 20-30 mcg/kg Term infant: 25-35 mcg/kg 1 month to 2 years: 35-60 mcg/kg 2-5 years: 30-40 mcg/kg 5-10 years: 20-35 mcg/kg >10 years: 10-15 mcg/kg TDD IV/IM: Preterm infant: 15-25 mcg/kg Term infant: 20-30 mcg/kg 1 month to 2 years: 30-50 mcg/kg 2-5 years: 25-35 mcg/kg 5-10 years: 15-30 mcg/kg >10 years: 8-12 mcg/kg Administer 50% of TDD initially; remaining 2 doses at 25% TDD q6-12h Maintenance dose PO: Preterm infant: 5-7.5 mcg/kg/d divided bid Term infant: 6-10 mcg/kg/d divided bid 1 month to 2 years: 10-15 mcg/kg/d divided bid 2-5 years: 7.5-10 mcg/kg/d divided bid 5-10 years: 5-10 mcg/kg/d divided bid >10 years: 2.5-5 mcg/kg qd Maintenance dose IV/IM: Preterm infant: 4-6 mcg/kg/d divided bid Term infant: 5-8 mcg/kg/d divided bid 1 month to 2 years: 7.5-12 mcg/kg/d divided bid 2-5 years: 6-9 mcg/kg/d divided bid 5-10 years: 4-8 mcg/kg/d divided bid >10 years: 2-3 mcg/kg qd |
| Contraindications | Documented hypersensitivity; digitalis-induced toxicity; AV block (without pacemaker); idiopathic hypertrophic subaortic stenosis; constrictive pericarditis |
| Interactions | Levels can be markedly altered by a number of medications; cholestyramine, metoclopramide, sulfasalazine, and chemotherapy all significantly lower digoxin levels; erythromycin, tetracycline, amiodarone, verapamil, quinidine, and quinine increase serum levels |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Dosage adjustment is required in patients with renal impairment; can cause cardiac arrhythmias; patients are predisposed to digoxin toxicity with hypokalemia, hypomagnesemia, hypercalcemia, and hypermagnesemia; CNS effects, such as drowsiness, and GI effects, such as nausea and vomiting, are some of the more common adverse drug reactions |
| Drug Name | Dopamine (Intropin) |
|---|---|
| Description | Adrenergic agonists are often used in the critical care setting for their rapid onset of action and rapid peak effect. They are, therefore, much easier to titrate to effect in acute settings. Their half-life is also much shorter than digoxin's, and their effects are rapidly lost when drug is discontinued. |
| Adult Dose | 1-20 mcg/kg/min continuous IV infusion; not to exceed 50 mcg/kg/min |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity; ventricular fibrillation |
| Interactions | Effects are prolonged and intensified by MAOIs, alpha- and beta-blockers, general anesthetics, and phenytoin |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Hypovolemia should be treated before infusion of this drug; administration through a central vein is recommended; do not use umbilical artery for infusion; if dosages >20 mcg/kg/min are required, a different agent should be considered (eg, epinephrine, dobutamine) |
These agents are used for management of right heart failure and pulmonary edema.
| Drug Name | Furosemide (Lasix) |
|---|---|
| Description | First-line drug for diuresis in newborns and infants and can be expected to be highly effective. It is a sulfonamide derivative that exerts its effects on the loop of Henle and distal renal tubule, thus inhibiting reabsorption of sodium and chloride. |
| Adult Dose | 10-200 mg PO/IV average dose; titrate to effect; doses as high as 600 mg/d may be used; continuous IV infusions may be more successful; usual maximum dosage approximately 0.4 mg/kg/h |
| Pediatric Dose | 1-2 mg/kg/dose PO/IV bid/tid/qid; titrate to effect; not to exceed 6 mg/kg/dose |
| Contraindications | Documented hypersensitivity; hypokalemia; renal failure |
| Interactions | Decreases effectiveness of oral hypoglycemic agents; may enhance effects of antihypertensives; may potentiate effects of succinylcholine; potentiates ototoxicity of aminoglycosides |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Inform patients of potential for photosensitivity; most popular strengths of digoxin and furosemide are white tabs of approximately equal size and may be confused in patients taking these medications on an outpatient basis; monitor serum potassium levels closely; may produce intravascular dehydration, severe hypokalemia, and significant hypochloremic metabolic alkalosis; may cause hyperuricemia; may produce deafness due to ototoxicity; administer oral dose with food or milk to decrease stomach upset |
| Media file 1: Echocardiogram showing the proximal chamber (PC) and distal chamber (DC) of the left atrium. The right atrium (RA), left ventricle (LV), and right ventricle (RV) are also shown.Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: ECG |
| Media file 2: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: ECG |
| Media file 3: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Image |
| Media file 4: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Image |
| Media file 5: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Image |
| Media file 6: This film shows the classic pattern of pulmonary edema associated with pulmonary overcirculation and pulmonary venous obstruction. The patient has an anomalous pulmonary venous connection that was only obvious after a pulmonary artery shunt. The particular radiograph is not of a patient with cor triatriatum, but the appearance of prominent pulmonary vascularity is the same. Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Video |
| Media file 7: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Video |
| Media file 8: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Video |
| Media file 9: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Video |
| Media file 10: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Video |
| Media file 11: Echocardiogram provided with permission from Guido Giordano, MD, Cardiovascular Department - Azienda Ospedaliera Cannizzaro, Catania, Italy, as shown at http://space.tin.it/scienza/guidogio/. | |
![]() | View Full Size Image | Media type: Video |
Article Last Updated: Jul 6, 2006