Presentation on theme: "Cor Triatriatum Seoul National University Hospital"— Presentation transcript:
1Cor Triatriatum Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery
2Cor Triatriatum 1. Definition 2. History A rare congenital cardiac anomaly in which the pulmonaryveins enter a proximal left atrial chamber separated from thedistal left atrial chamber by a diaphragm in which there areone ore more restrictive ostia.* Cor triatriatum sinister, Cor triatriatum dexter2. HistoryChurch : 1st description in 1868Borst : The term of cor triatriatum in 1905Miller et al : Angiographic diagnosis in 1964Ostman-Smith : Description by Echo. In 1984Vineberg & Gialloreto : 1st surgical correction in 1956
3Cor Triatriatum Pathophysiology Persistence of a common pulmonary vein forms an accessory left atrial chamber that communicates with the true left atrium via the restrictive fibromuscular diaphragm.Pulmonary venous return is restricted, resulting in pulmonary venous congestion and hypertension, right ventricular hypertrophy, and congestive heart failure.
5Cor Triatriatum 3. Types 1. Prevalence 2. Embryology 0.1-0.4% of CHD 1) Communication with LA(classic)a. With intact atrial septumb. CPVC communicating with RAc. Atrial defect into lower chamber2) No communication with LA(imperforate diaphragm)Drainage into RADrainage into coronary sinusDrainage into systemic vein1. Prevalence% of CHDEqual frequency in both sexIsolated : 33-50%2. Embryology* Dorsal eventration of commonatrium (CPV), enlarges to joinpulmonary segment ofsplanchnic plexus.* Malformations result fromabnormal growth or regressionof common pulmonary vein.
6Pulmonary Venous Connection Patterns of malformation1. Total anomalous pulmonary venous drainageThe common pulmonary vein fails to establish communicationwith pulmonary venous plexus, then pulmonary vein tosystemic venous connection will persist.2. Atresia of common pulmonary veinThe common pulmonary vein is obliterated after the venouschannels have disappeared.3. Cor triatriatum sinisterAfter connection between atrium and confluence of pulmonaryveins accomplished by common pulmonary vein, abnormalincorporation into left atrium causes stenosis or obstruction.
7Morphology of Cor Triatriatum 1. Classical morphology* Thick common pulmonary vein chamber & thin distal LA chamber* One or more openings, thick and fibromuscular, or tubular* RV enlarged due to Lt. to Rt. shunt* LV usually normal or small* Foramen ovale usually patent and stretched* PAPVR may coexist2. Relationship to a left SVC* Frequently than other type of CHD,* Theory of impingement of SVC on developing LA3. Associated anomalies* PAPVR, TAPVR* Unroofed coronary sinus with Lt SVC* VSD, COA, AV canal, TOF
10Clinical Features & Diagnosis 1. Patients with small opening present with evidence of LCO( pallor, tachypnea, poor peripheral pulse, growth failurewith pulmonary venous hypertension)2. When there is associated Lt. to Rt. Shunt, evidence ofpulmonary overcirculation and venous obstruction maybe present in X-ray, and right ventricular enlargementis prominent3. Signs and symptoms of pulmonary venous hypertension4. Diagnosis can be suspected by echocardiography, MRI
11Cor Triatriatum Natural History 1. Rare congenital cardiac anomaly 2. Natural history depends on effective size ofhole in the partition between the commonpulmonary venous chamber and left atrium3. Mostly, the hole is severely restrictive andabout 75% die in infancy without treatment4. When communicates with right atriumthrough ASD, the prognosis is better( depend on the size of hole).
12Techniques of Operation 1. Typical cor triatriatumDiaphragm is excised to make an openingas large as possible and the opening inthe atrial septum closed.2. Atypical cor triatriatumCombination of typical repair, andanomalous pulmonary venous connection.
13Operative View of Cor Triatriatum Excised daiphragmDiaphragmOpening
14Cor Triatriatum Operative Results 1. Early death Uncommon, but occurs in critically ill withinadquate myocardial management2. SurvivalApproaches that of general population andgood functional result3. Complications* Restenosis due to inadequate resection* Pulmonary vein stenosis
15Indications for Operation 1. Urgent indication for restrictive aperture2. Operation is necessary in the 1st year of life3. In older patients with chronic symptom,operation is also urgently indicated.4. In complex cor triatriatum, operation isindicated on an urgent basis.
16Cor Triatriatum Dexter 1. DefinitionA rather unusual congenital cardiac malformation in which apersistent right valve of sinus venosus subdivides right atrium2. HistoryRokitansky : 1st description in 18753. EmbryologyRight horn of sinus venosus is incorporated into right atrium,giving rise to orifice of the SVC and IVC.At one point the right valve of sinus venosus nearly divides rightatrium, the valve regresses and moves caudally, leaving thecrista terminalis, and the valve of IVC (Eustachian) andcoronary sinus (Thebesian).
17Cor Triatriatum Dexter Clinical Features1. PathologySome minor degree of septation of right atrium by theremnant of right valve of sinus venous is well tolerated,and a prominent Eustachian valve is common finding.2. Clinical presentation* Somewhat variable* Asymptomatic in vast majority* Recurrent supraventricular tachycardia* Right heart failure in symptomatic patients* Cyanosis in the presence of ASD