Cor Triatriatum Sinister (Divided Left Atrium): Histopathologic Features and Clinical Management.

BACKGROUND: Cor triatriatum sinister (CTS), or divided left atrium, is a rare congenital cardiac disease in which the left atrium is divided into 2 chambers by a fibromuscular diaphragm that will cause blood flow obstruction to the left ventricle. Recent animal studies suggested the role of hyaluronidase-2 (HYAL-2) deficiency as a risk factor for developing CTS. The histopathologic features of this diaphragm and our surgical experience with the management of this disease are reviewed. METHODS: Ten patients underwent surgical correction of CTS between 2010 and 2018. All patients had complete clinical and imaging evaluation. The fibromuscular diaphragms were histologically evaluated with myosin, troponin, vimentin, smooth muscle actin, and HYAL-2 to characterize the structure of the CTS diaphragm. RESULTS: All patients underwent excision of CTS diaphragm using cardiopulmonary bypass with no early mortality. Most patients had the classic form of CTS in which the diaphragm separates the pulmonary and the vestibular chambers with no atrial septal defect. The histologic studies demonstrated the presence of fibrous, mesenchymal cells, along with cardiac muscle cells, at the site of membrane attachments. HYAL-2 enzyme was expressed in the CTS diaphragm. CONCLUSIONS: Surgical repair of CTS provides satisfactory results with low risk of death. Our histologic studies revealed the cellular composition of the CTS diaphragm. HYAL-2 deficiency may not explain the pathogenesis of CTS, and further studies are needed to evaluate the complex mechanisms involved in the development of this disease.

Bidirectional flow across a perforate cor triatriatum dexter in a dog with concurrent pulmonary, tricuspid, and mitral valve dysplasia.

A 10-week-old male intact mixed breed dog presented for evaluation of suspected right-sided congestive heart failure. Echocardiographic imaging revealed a perforate cor triatriatum dexter (CTD), along with pulmonary valve stenosis and tricuspid and mitral valve dysplasia. In typical CTD cases, there is unidirectional blood flow across the dividing membrane, from the caudal into the cranial right atrial chambers. Owing to right-sided pressure alterations caused by the concurrent valvar defects, color Doppler imaging demonstrated bidirectional flow across the CTD membrane.

Surgical correction of cor triatriatum sinister in the paediatric population: mid-term results in 15 cases.

OBJECTIVES: Cor triatriatum sinister (CTS) is a rare developmental cardiac abnormality resulting in left ventricular inflow obstruction. In this report, we aimed to present our mid-term results of early childhood patients operated for CTS and associated cardiac abnormalities. METHODS: We enrolled 15 patients with CTS who were operated by a single surgeon between 2001 and 2013. A retrospective analysis was performed in order to determine the demographics, operative and postoperative results of the patients. The median age was 14 months and the median body weight was 8.2 kg at the time of operation. RESULTS: Fourteen patients had concomitant cardiac pathology. Three of the patients had atrial septal defect and 1 of the patients had partial abnormal pulmonary venous connection, whereas 4 of the patients had both. In 2 cases of complete atrioventricular septal defect, 1 case with ventricular septal defect, 1 case with patent ductus arteriosus, 1 case with double outlet right ventricle and another case with tetralogy of Fallot, complete repair was performed together with membrane resection in the left atrium (LA). The mean preoperative left atrial gradient was 13.3 mmHg, whereas the mean LA pressure at the first postoperative year was 4.2 mmHg. There was 1 case with early mortality due to septic multiorgan failure secondary to pneumonia. CONCLUSIONS: CTS is a rare congenital cardiac anomaly in which the results of the corrective surgery are encouraging. Early and long-term outcomes may be variable according to the associated congenital heart defects.

Cor triatriatum sinistrum in childhood. A single institution's experience.

INTRODUCTION: Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients. OBJECTIVE: To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome. METHODS: Hospital databases were searched to identify patients with CTS who were diagnosed between 1954 and 2005. Medical records with demographic data, clinical evaluation, diagnostic approach, interventions and autopsy results were reviewed. RESULTS: Between 1954 and 2005, 82 patients (43 female children, 52%) with CTS were diagnosed at the institution. Patients were born between 1951 and 2004, and the median age at presentation was eight months (range one day to 16.1 years). The majority of patients (77%) presented with associated cardiac lesions. Of 82 patients, 57 (70%) underwent resection of the fibromuscular diaphragm, 14 (17%) did not require surgery and 11 (13%) did not survive the time to intervention. Nineteen patients (23%) died a median of two months (range one day to 5.5 years) after presentation. Nine of these patients (11%) died before surgery was attempted. Most patients were completely asymptomatic at the time of the last follow-up at a median of three years (range one day to 18 years). Echocardiography significantly facilitated the diagnosis and subsequent management of patients with CTS. CONCLUSIONS: Despite the diversity in presentation and complexity of associated lesions, the outcome for patients with CTS is favourable.

Combination of cor triatriatum sinistrum and hypoplastic left heart syndrome in Meckel-Gruber syndrome: a case report.

Meckel-Gruber syndrome (MKS) is a fatal, autosomal recessive disorder characterized by malformation of central nervous system, particularly occipital encephalocele, bilateral renal dysplasia, and polydactyly. However, the clinical findings of this syndrome encompass various organ abnormalities as a result of genetic heterogeneity. The associated heart anomaly in MKS is inconstant. Its prevalence is rare and no striking or specific cardiac defects have been documented. We present a case of MKS with combined cor triatriatum sinistrum (left atrium divided into upper and lower compartment by a thin membrane) and hypoplastic left heart syndrome (underdeveloped mitral valve, left ventricle, and aorta) in a 33-week male fetus that was ultrasonographically detected and confirmed by autopsy. In addition to the cardiac defects, the patient was found to have postaxial polydactyly of 4 extremities, Dandy-Walker malformation, bilateral renal cystic dysplasia, and hepatic plate malformation. To the best of our knowledge, this is the first time that a combination of cor triatriatum sinistrum and hypoplastic left heart syndrome in MKS has been reported in the literature.

[Myxoma of the cor triatriatum].

We present a rare case of myxoma in a patient with cor triatriatum. This pathology was symptomless and was revealed at transthoracic echocardiographic examination in a patient with ischemic heart disease. The patient was successfully subjected to surgery which consisted in removal of the tumor together with accessory membrane to which myxoma was attached and reconstruction of the atrial wall. In international medical literature we found no communication on the combination of myxoma with cor triatriatum.

Radical repair of cor triatriatum with partially unroofed coronary sinus and persistent left superior vena cava 35 years after operation for tetralogy of Fallot.

We report a rare case of simultaneous repair for Cor triatriatum (CT), partially unroofed coronary sinus (CS) with persistent left superior vena cava (PLSVC), moderately severe tricuspid regurgitation, and atrial flutter 35 years after radical operation for tetralogy of Fallot (TOF). A 40-year-old woman underwent patch closure of atrial septal defect as to drain blood from left SVC to right atrium (RA), excision of diaphragm in left atrium (LA), tricuspid annuloplasty, and cryoablation. Postoperative course was uneventful with successful anatomical correction.

Late presentation of cor triatriatum with persistent levoatrial cardinal vein.

An asymptomatic 10-year-old boy presented with reduced exercise tolerance and an echocardiographic diagnosis of cor triatriatum. Transthoracic and transesophageal echocardiography failed to reveal the persistent levoatrial cardinal vein discovered at surgery. In patients with late presentation of cor triatriatum with severe mitral inflow obstruction and a small patent foramen ovale, an alternative communication between the posterior collecting chamber and the systemic venous circulation should be sought with alternative imaging techniques.

Supramitral valve obstruction from hypertrophied native atrial tissue as a complication of orthotopic heart transplantation.

An acquired form of cor triatriatum, resulting in significant supramitral valve obstruction, complicating a pediatric orthotopic heart transplantation was diagnosed by transesophageal echocardiography. Surgical resection of the hypertrophied atrial septum and atrial wall corrected the anatomic obstruction and improved the hemodynamics.

[Growth in children with the exudative enteropathy syndrome due to a congenital heart defect--cor triatriatum dextrum]. / Rust u dítete se syndromem exsudativní enteropatie na podklade vrozené srdecní vady--cor triatriatum dextrum.

Retarded growth in a child can be the sign of serious chronic disease. The authors present an account of a six-year-old boy where growth retardation persisted at least from the age of three. During this period his height dropped from the zone between the 25th and 50th percentile into the zone between the 3rd and 10th percentile. From the clinical point of view a large abdomen, loose stools and hypocalcaemia with tetany were striking, as they were moreover refractory to vitamin D2, calcitriol and calcium administration by the oral route. The authors revealed severe hypoproteinaemia, a 150 times increased value of alpha-1-antitrypsin in faeces, and exudative enteropathy syndrome was diagnosed. The cause was venous congestion due to a rare heart disease--cor triatriatum dextrum. The septum in the right atrium was resected. Immediately after surgery the consistency and frequency of stool decreased. Calcaemia and plasma protein levels reached normal levels within two months. A growth spurt of 11 cm/year followed. Fifteen months after operation the patient's height reached almost the 50th percentile.

[Cor triatriatum--a rare cause of pulmonary hemosiderosis]. / Cor triatriatum--seltene Ursache einer Lungenhämosiderose.

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.

[A surgical case of cor triatriatum].

A case report of a variant type of cor triatriatum was presented. The patient was critically ill when she was admitted to our hospital. An emergent operation was performed under the cardio-pulmonary bypass. The right atrium was incised, then the interatrial septum was incised. Inspection revealed an abnormal membrane in the left atrium. All the pulmonary veins were drained into the accessory chamber. The left atrial appendage was also connected with the accessory chamber. The membrane was not including the mitral valve tissue. It was resected completely. Postoperative course was uneventful.

Persistencia da valva direita do seio venoso septando parcialmente o atrio direito: relato de caso / Persistence of the right valve of the sinus venosus parthy septing the right atrium

E descrito um caso de persistencia da valva direita do seio venoso (chamado coracao triatal direito). Coexistia estenose pulmonar grave e forame oval patente. Apesar destas anomalias o paciente viveu ate 25 anos, sem queixas importantes. Faleceu em decorrencia de complicacoes tromboembolicas pulmonares. Esta anomalia foi detectada em vida atraves de ecodopplercardiografia; sao descritos em detalhes os achados necroscopicos do coracao.

Cor triatriatum. Clinical presentation and operative results.

From November 1973 to January 1988, 15 patients with cor triatriatum underwent surgical correction at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. Their ages ranged from 15 days to 48 years. Eight patients (53%) were younger than age 1 year. Evidence of congestive heart failure was present in five infants, whereas three infants, three children, and two adults initially had signs of pulmonary venous obstruction, and two children had a heart murmur only. Five patients had cor triatriatum alone; in ten cases there also was an atrial septal defect. Associated anomalies in four patients included left superior vena cava, ventricular septal defect and left superior vena cava, partial anomalous pulmonary venous connection, and bilateral partial anomalous pulmonary venous connection. Cor triatriatum was repaired with the aid of cardiopulmonary bypass in all patients. Excision of the membrane was accomplished with a right atrial approach in 13 patients and a left atriotomy in one patient. Both atria were opened in one case. All associated anomalies were simultaneously corrected. Three patients (20%) died early after operation. Among the 12 survivors, no late events have occurred, and all of them are presently in New York Heart Association functional class I. A recent echocardiogram shows absence of residual obstruction or shunt and good development of the left cardiac chambers.

Anomalies of the systemic venous return: a review.

Congenital anomalies of the systemic venous connection to the heart represent a rather wide and heterogeneous group of malformations, whose physiological consequences may vary from nil to the most severe form of systemic arterial desaturation. The malformations may be summarized as follows: (1) Left superior vena cava connected to the coronary sinus, interrupted inferior vena cava and absent right superior vena cava that do not indicate surgical repair 'per se', but require some technical attention during open heart surgery performed for other anomalies; (2) Left superior vena cava connected to the left atrium, due to incorporation of the coronary sinus into the left atrial cavity, resulting in a right-to-left-shunt; (3) Right superior vena cava or inferior vena cava draining into the left atrium, both are extremely rare and require treatment for the ensuing right-to-left shunt; (4) Total anomalous systemic venous connection to the left atrium, usually combined with atrial isomerism and other very complex heart malformations; (5) Cor triatriatum dexter, which has been frequently diagnosed as an anomalous venous connection for its similar hemodynamic consequences. Such anomalies are reviewed with particular respect to their surgical implications.

[Cor triatriatum. Anatomical forms and surgical treatment of 6 consecutive cases]. / Coeur triatial. Formes anatomiques et traitement chirurgical de 6 cas consécutifs.

Six cases of cor triatriatum documented and operated on at Henri-Mondor hospital between 1980 and 1984 are reported. Ages at the time of surgery ranged from 8 months to 57 years. Four of the 6 patients presented with pulmonary hypertension. Anatomic types consisted of 4 diaphragmatic types and 2 more complex malformations. Associated lesions were present in all but one patients. They consisted mostly of interatrial defects (depending on their position with regard to the intra-atrial membrane, they play a determinant role in the symptomatology) and of a left superior vena cava (which might play a role in the embryogenesis of the malformation). Except for one early postoperative death, results of surgery were excellent for all patients, with a mean follow-up of 5 years. This series, compared with the literature, allows precising the embryologic and anatomic aspects of the malformation and the surgical techniques in complex types.

Cor triatriatum dexter with anomalous pulmonary venous drainage and sinus venosus atrial septal defect.

The atypical location of a membrane in a case of cor triatriatum dexter prompted successful repair of accompanying anomalies, with in situ use of the membrane. The anatomy is described with a review of the literature.