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We report on a 49-year-old fitness trainer, who was admitted to our hospital after cardiac arrest due to ventricular fibrillation. Return of spontaneous circulation was achieved after immediate cardiopulmonary resuscitation. Coronary angiography could exclude coronary artery disease. Echocardiography demonstrated the presence of apical hypertrophic cardiomyopathy, associated with cor triatriatum sinister. Cardiac magnetic resonance imaging additionally showed marked myocardial fibrosis. The patient underwent placement of an implantable cardioverter-defibrillator and was subsequently discharged for rehabilitation in good condition.

We report on an asymptomatic 2 1/4 year old girl. Echocardiography revealed cor triatriatum with a membrane separating the left atrium. Transesophageal echocardiography demonstrated 3 large perforations of the intraatrial membrane. Using Doppler sonography, no pressure gradients across these orifices were identified. Obstruction of the intraatrial membrane and pulmonary hypertension were excluded by cardiac catheterization. We therefore decided against surgical resection of the membrane and to follow the patient noninvasively by echocardiography. Follow-up over 18 months revealed no development of an obstruction across the intraatrial membrane. Our case shows that immediate surgical correction is not necessary in all patients with cor triatriatum. Conservative management of these patients requires full evaluation of the hemodynamics and careful follow-up examinations by echocardiography.

We report the case of a 77 year old man, who was hospitalized in the final stadium of his congenital disease. Following a pneumonia in the right lung the respirator therapy was needed. Later the patient died by a chronic right heart failure.

Cor triatriatum dexter is a rare congenital malformation in which a membrane divides the right atrium into two chambers. The membrane represents a persistence of the right sinus venosus valve (RSV). Normally the RSV regresses between the 9th and 15th week of gestation, as the cephalic portion forms the crista terminalis and the caudal portion develops into the Eustachian and Thebesian valve. Any failure in the regression process may result in remnants of RSV as a simple muscle bar, a Chiari-network or a fenestrated or unfenestrated membrane (cor triatriatum dexter). We describe a patient with cor triatriatum dexter in whom diagnosis was made several years after successful valvulotomy procedure for severe congenital valvular pulmonary stenosis.

Pneumological examinations including open lung biopsy performed on a male patient of 30 years of age suffering from severe respiratory distress that disabled him, as well as from massive recurring attacks of hemoptysis, resulted in suspicion of idiopathic pulmonary hemosiderosis (also known as Ceelen-Gellerstedt's syndrome). Diagnosis of cor triatriatum followed by surgery was arrived at only after a pulmonary oedema had developed and after other rare cardiac diseases had been considered. This rare congenital malformation--which occasionally becomes clinically manifest only in the adult--should be suspected in differential diagnosis of respiratory distress and a sometimes also life-threatening hemoptysis. Echocardiography is the diagnostic method of choice in this regard.

We report on two patients (a 52-year-old female and a 32-year-old male) with cor triatriatum sinistrum, both clinically inconspicuous. The former had undergone strenuous athletic training without difficulty for eight years of her youth. In both cases, cor triatriatum sinistrum was found by chance during transthoracic echocardiography undertaken for hypertension. The exact insertion-site of the left atrial membrane only became apparent by using biplane transoesophageal echocardiography. Neither of the patients showed associated cardiac malformation. Hemodynamics were evaluated by pulsed waved Doppler analysis of pulmonary venous flow profile; no signs of inflow obliteration into the left atrium were found in either case.

Cor triatriatum is a rare cardiac malformation occurring in about 0.1% of all congenital heart disease. As symptoms are usually present soon after birth, diagnosis in adulthood is extremely rare. We describe a case of a 33-year-old asymptomatic woman who was referred for echocardiographic examination because of a postural variable systolic click. The anatomy of the malformation and the hemodynamics of the left atrium were accurately assessed by means of conventional transthoracic Doppler echocardiography. The antero-inferior course of the intraatrial membrane inserting proximal to the left atrial appendage was documented in multiple perpendicular planes of examination. All pulmonary veins drained correctly into the proximal compartment of the left atrium. Additionally, a small atrial septal defect communicated between the distal compartment of the left atrium and the right atrium. Using pulsed wave Doppler, no site of pressure gradient could be detected between the two compartments of the left atrium. Thus, conventional transthoracic Doppler echocardiography represents an adequate tool for assessing cor triatriatum in adults. The patient refused transesophageal echocardiography.

Cor triatriatum dextrum is a rare malformation of the right atrium usually without typical signs or symptoms. Today the intra vitam diagnosis of C.t.d. is done by echocardiography, MR and angiocardiography. Once the diagnosis is established, surgical correction of this anomaly is possible in symptomatic patients.

Cor triatriatum dextrum is a rare malformation of the right atrium usually without typical signs or symptoms. Today the intra vitam diagnosis of C.t.d. is done by echocardiography and angiocardiography. Once the diagnosis is established, surgical correction of this anomaly is possible in symptomatic patients. When the presence of an abnormal membrane in the right atrium is not recognized before operation, the outcome may be unsatisfactory.

Cor triatriatum was diagnosed in a 32-year-old woman (Case 1) and a 36-year-old man (Case 2). The definitive diagnosis in Case 1 was made by transthoracic 2-D echocardiography, in Case 2 (after a chance finding) only after additional transoesophageal echocardiography. Colour Doppler echo in Case 1 provided information on the number and localization of membrane openings, while in Case 2 simultaneous measurement of maximal flow velocity and normal right-sided pressures indicated that the anomaly was haemodynamically insignificant owing to the size of the central opening in the membrane (maximal diameter 2.1 cm). In Case 1, abnormal haemodynamic findings on right-heart catheterization provided the indication for surgery and the membrane was successfully removed. Postoperatively the patient was much improved and cardiac catheterization demonstrated normal values.