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PubMed2006

[Assessment of the characteristics of echocardiography in pediatric patients with total anomalous pulmonary venous connection].

OBJECTIVE: To assess the accuracy of echocardiography in diagnosis of total anomalous pulmonary venous connection (TAPVC). METHODS: A combination of suprasternal, parasternal, subcostal and apical views were employed to diagnose TAPVC and to trace the course of the anomalous pulmonary venous connection, the direction of the inter-atrial shunt, enlargement of right atrium (RA) and right ventricle (RV), superior and inferior vena cava. All pediatric patients underwent surgical repair. The results of echocardiography were compared with surgical findings. RESULT: A total of 28 consecutive pediatric patients with suspected TAPVC were included in this study. The TAPVC diagnosis was confirmed in 26 cases after surgery, partial anomalous pulmonary venous connection (PAPVC) in one case, and Cor Triatriatum and possible TAPVC in another. The diagnostic accuracy of TAPVC by echocardiography in the study was 92.86%. There were 17 supracardiac TAPVC, 11 intracardiac TAPVC. In all patients, enlargement of the RA and RV, inter-atrial right-to-left shunt via atrial septal defects were documented in parasternal and subcostal views. Common pulmonary vein or four pulmonary vein direct to RA or via coronary sinus to RA were the draining sites of intracardiac TAPVC. The enlargement of left innominate vein-right superior vena cava draining to RA was seen in supracardiac TAPVC. CONCLUSION: A combination of suprasternal and subcostal multi-views in echocardiography can increase the diagnostic accuracy of TAPVC in pediatric patients.

Abnormalities, Multiple↗
PubMedDate not supplied

Supravalvular mitral stenosis in a cat.

A 3-year-old, 4-kg, castrated male domestic shorthair cat presented with signs of progressive respiratory distress. Thoracic radiographs showed pulmonary edema and pleural effusion. Echocardiography revealed a perforate membrane immediately above the mitral valve that divided the left atrium into proximal and distal chambers. The left auricle was proximal to the dividing membrane and connected to the markedly enlarged proximal left atrial chamber, consistent with the diagnosis of supravalvular mitral stenosis (SMS). Position of the obstructing membrane relative to the left auricle distinguishes SMS from cor triatriatum sinister (CTS). In CTS, the left auricle is distal to the dividing membrane and connects to the distal left atrial chamber.

Animals↗
PubMed2000

[Neurologic outcome in infants surgically treated for congenital cardiopathy: preliminary data].

INTRODUCTION: Both surgical techniques for correction of congenital heart diseases (CHD) and intraoperatory neurologic protection improved during the last 20 years. Nevertheless cardiac surgery is still a risk for neurologic morbidity. METHODS AND PATIENTS: Analysis of the postoperative neurologic status of infants younger than 6 months who underwent cardiac surgery from January 1998 to December 1999. We reviewed the EEG tracings, cranial ultrasound reports (CUS) and CT scans of 48 patients. Diagnoses were: ventricular septal defect = 15, Fallot (TOF) = 9, patent ductus arteriosus (PDA) = 5, coarctation of aorta = 4, atrio-ventricular septal defect = 4, transposition of great arteries (TGA) = 3, hypoplastic left heart syndrome = 2, pulmonary atresia = 2, total anomalous pulmonary veins drainage = 2, double outlet right ventricle = 1, cor triatriatum = 1. Mean age (range) at intervention was 54 days (2-150), 44 infants (91.7%) survived at follow-up: 23 EEG, 22 CUS and 2 CT were performed in the recent postoperative. Among survivors 5/44 had neurologic complications. EEG was altered in 4: two of them (1 TOF, 1 TGA) had pathologic CUS and CT as well (ischemic pattern in the former, atrophy in the latter). Finally a preterm newborn with PDA had mild abnormalities at CUS. After a mean follow-up of 16 +/- 6 months 3/5 patients had mild-to-moderate psychomotor delay and 2 recovered. CONCLUSIONS: According to our preliminary data the prevalence of neurologic complications in infants who undergo cardiac surgery seems to be low. The pathological findings of the recent postoperative seem to recover up to normalization in some cases at mid-term follow-up. As expected, permanent complications effect more often complex CHD. Further follow-up studies to school age will be mandatory to check the very final results of cardiac surgery performed during early infancy.

Electroencephalography↗
PubMed1979

[Total anomalous pulmonary venous return into the coronary sinus. Apropos of 1 case with a difficult diagnosis and surgical success].

The case of an 8 year-old boy with total anomalous pulmonary venous return into the coronary sinus is reported. The coronary malformation comprised a considerable dilatation of the coronary sinus which made diagnosis very difficult even at open heart. The surgical success was obtained by resecting the interatrial septum and partitioning the atrium. The anatomical diagnosis of this condition and the malformations with which it could be confused are discussed: TAPVR into the right atrium, cor triatriatum. The two parts of the coronary sinus, confluence of the cardiac veins and the left horn of the sinus venosus, are described.

Child↗
PubMed2002

Giant eustachian valve with echocardiographic appearance of divided right atrium.

The eustachian valve is an embryologic remnant of the valve of the inferior vena cava. It may be prominent in some individuals, but the echocardiographic appearance of a divided right atrium, as the consequence of a large eustachian valve, is extremely rare. Herein we describe an unusual giant eustachian valve with an echocardiographic appearance of a septal structure dividing the right atrium into 2 separate chambers. This abnormality should be differentiated from cor triatriatum dexter, a very rare cardiac malformation for which it could be mistaken.

Echocardiography↗
PubMed1991

Pulmonary edema due to extreme left atrial compression.

Pulmonary edema is a common concomitant of valvular heart disease and ventricular dysfunction. In addition, left atrial dysfunction due to thrombus, myxoma, or cor triatriatum can produce the same clinical picture. We encountered a patient with intractable pulmonary edema secondary to obliteration of the left atrial cavity by an extrinsically compressing lung tumor. We believe extrinsic compression of the left atrium caused impaired left atrial filling, leading to pulmonary venous hypertension and pulmonary edema.

Atrial Function, Left↗
PubMed1990

[Congenital heart defects and abnormalities newly detected with echocardiography in adolescents and adults].

In 12,576 consecutive Doppler-echocardiographic studies (DE) performed on 11,450 patients over 3 years (1987-1989), 183 newly diagnosed congenital heart abnormalities were detected in 161 patients. The age of the 161 patients (86 female, 75 male) ranged from 16 to 84 years (median 34.5). The highest incidences of pathologies were those of ASD II (29%) and VSD (11%). With decreasing frequency the following abnormalities were diagnosed: pulmonary valve disease (8%), interatrial septal aneurysms (7%), membranous subvalvular aortic stenosis (5%), anomalous pulmonary venous connections (4%), atrioventricular canal defects (4%), persistent left superior venae cavae (4%), patent ductus arteriosus (4%), aneurysms of the membranous septum (4%), and sinus venosus defects (3%). Less common findings were coarctation of the aorta (2.5%), persistent sinusoids (2.5%), arteriovenous fistulas (2.5%), congenital LV-aneurysms (2.5%), Ebstein's anomalies (1%), anomalies of the papillary muscles (1%) and RV-dysplasias (1%). Rare findings were a cor triatriatum, a tetralogy of Fallot, a partial defect of the pericardium, a pulmonic atresia with VSD, an isolated cleft of the tricuspid valve, and finally a connection of a hepatic vein to the right atrium. In conclusion, we found an incidence of 1.4% in newly diagnosed congenital heart disease in adolescents and adults undergoing DE. 32 of 159 patients (20%) were referred to surgery, while endocarditis prophylaxis was indicated in 45%.

Adolescent↗
PubMed1989

[[Doppler echocardiography in the diagnosis of tri-atrial heart in adults].

The authors report a case of isolated and well tolerated cor triatriatum in a 38-year old man without significant history admitted for aetiological diagnosis of atrial fibrillation. Echocardiography displayed the left intra-atrial septum and the openings of the 4 pulmonary veins in the proximal chamber, but there was no evidence of associated anomaly. Colour-coded doppler examination visualized a single systolo-diastolic trans-septal jet. As the maximum velocity of the jet at pulsed doppler velocimetry was 1.1 m/s, the maximum instantaneous gradient could be estimated at 5 mmHg. The mean gradient was estimated at 2.6 mmHg, which showed that the ostium caused little stenosis. All these data were confirmed by cardiac catheterization. Since the malformation was well tolerated, both functionally and haemodynamically, it was decided not to operate.

Adult↗
PubMed1988

Divided right atrium (prominence of the eustachian and thebesian valves).

Division of the morphologically left atrium (cor triatriatum) is a recognized clinical and surgical entity. Division of the right atrium (prominence of the eustachian and thebesian valves) is recognized pathologically, but is rare. It is unusual for this entity to be diagnosed during life. Stimulated by our experience with two patients seen at operation, one with an obstructive spinnaker-like formation and the other with a partitioned right atrium in the setting of pulmonary atresia, we reviewed the specimens in the heart museum of Children's Hospital of Pittsburgh that had prominence of the eustachian and thebesian valves. We identified 14 such hearts, which could be divided into two groups. In the first group, comprising six hearts, the valves were prominent in the form of Chiari networks and were of no functional significance. The valves were more extensive in the other eight hearts and partitioned the right atrium. In two of these, the valves themselves were the impediment to flow through the right side of the heart. In the other six, there was either atresia or severe stenosis along the right-sided pathways so that, after birth, the prominent valves retained their role during fetal life; namely, to deflect inferior caval venous return across the atrial septum to the left atrium. The partitions in these latter hearts would be of functional significance only if it were necessary to perform a Fontan procedure, when they might obstruct flow through an atriopulmonary (or atrioventricular) anastomosis.

Female↗
PubMed1986

Morphogenesis of the venous valves in the rat heart. I. Light and scanning electron microscopy.

Hearts from rat embryos ranging from 12.5 days gestation through birth were studied with light and scanning electron microscopy. Both valves of the sinus venosus are very prominent structures in the right atrium of the developing rat heart. The right venous valve is large and undulating and extends to the right atrioventricular canal. Many nodules are apparent on the surface of this valve by the thirteenth day. These nodules decrease in number as development progresses. The left venous valve is considerably smaller than the right. Neither valve shows signs of regression, and just prior to birth, both valves are very prominent. The right valve is so large that it almost divides the right atrium into two chambers. Based on these observations the rat may serve as a model for cor triatriatum dexter.

Animals↗
PubMed1986

Rare primary sarcomas of the heart.

Three patients with primary malignant cardiac neoplasms are described. All tumors were intracavitary myxomatous masses of the left atrium. Preoperative clinical diagnostic techniques did not indicate malignancy, but suggested mitral stenosis, cor triatriatum, and cardiac myxoma. Grossly, the tumors were sessile rather than pedunculated, and they invaded the underlying structures. Microscopically, although the tumors resembled benign cardiac myxomas, they exhibited mitotic activity and areas of necroses. Ultrastructural examination revealed a spectrum of differentiated mesenchymal cells that lacked the maturation features of cardiac myxoma cells. These gross, microscopic, and ultrastructural features, which suggested the tumors' malignant potential, should help in the early recognition and management of similar tumors.

Adult↗
PubMed1979

[Echo-angiocardiographic correlations in congenital abnormalities of the mitral valve (author's transl)].

Echocardiographic findings satisfactorily compared with angiocardiographic data in six infants affected by congenital abnormalities of the mitral valve. Peculiar echocardiographic features were as follows: a) mitral stenosis: reduced E-F slope of the anterior mitral leaflet, paradoxical diastolic movement of the posterior leaflet, enlargement of the left atrium; b) "parachute mitral valve": small, dysmorphic, stiff mitral valve, with an extra echo projecting between those of the two leaflets; c) "cor triatriatum": enlarged left atrium whose cavity is divided in two portions by an extra echo; d) "hypoplastic left heart syndrome": the mitral leaflets are thin, short. The ventricular cavity and the diameter of the aorta are small; the right ventricle and the pulmonary artery are on the contrary markedly dilated.

Angiocardiography↗
PubMedDate not supplied

Successful conversion of fetal atrial flutter with digoxin: report of one case.

A fetus suffering from tachycardia of more than 200 beats per minute was found to have an atrial flutter with two-to-one atrioventricular conductional block by the fetal ultrasonogram at 33 weeks of gestation. The tachycardia was successfully converted to sinus rhythm with maternal digitalization. The heart rate stayed in a normal sinus rhythm after the baby was born, however, an incomplete form of cor triatriatum was noted on the echocardiogram examination.

Adult↗
PubMed1989

Umbilical catheter masquerading at echocardiography as a left atrial mass.

The case of a newborn who had an umbilical catheter inserted in the intensive care unit is discussed. This catheter was meant to be inserted into the umbilical artery, but was instead inadvertently inserted into the umbilical vein. It crossed a patent foramen ovale into the left atrium. This fact was not known at the time of echocardiographic evaluation. At echocardiography, abnormal echoes within the left atrium were seen. Findings were typical for a catheter within the heart, and the umbilical catheter was subsequently withdrawn. There is a potential for mistaking the echocardiographic findings for a left atrial abnormality and echocardiographers should be alert to this possibility.

Catheterization, Peripheral↗
PubMed1996

Uncommon etiologies of atrial fibrillation.

A newly diagnosed atrial fibrillation warrants a full investigation of the etiopathogenesis of this common arrhythmia. In the adult population, the most frequently associated conditions are systemic hypertension, coronary artery disease, mitral valvulopathy, congestive heart failure, and hyperthyroidism. Nevertheless, more infrequent and even rare, yet correctable, etiologies should not be overlooked. We describe three patients who presented to our hospital with a first episode of atrial fibrillation and who subsequently were demonstrated to have very unusual cardiovascular pathologies subtending this common arrhythmia. In all three cases, trans-esophageal echocardiography was instrumental in reaching an accurate diagnosis that was later confirmed at surgery.

Adult↗
PubMed2002

Divided right atrium associated with extensive coronary vein abnormalities.

This report describes a case of a divided right atrium associated with coronary vein abnormalities. An 18-year-old woman who had a past history of surgery for repair of a divided right atrium and atrial septal defect developed exertional dyspnea 8 years after the surgery. Selective coronary angiography showed a dilated right coronary artery with a fistulous communication to the right atrium, tortuous coronary veins draining directly into the cardiac chambers, and the absence of the coronary sinus. Embryologically, regression failure of the right venous valve is hypothesized to have created both the divided right atrium and extensive coronary vein abnormalities.

Adolescent↗
PubMedDate not supplied

Persistent eustachian valves in infants: course and management in symptomatic patients.

Although commonly found on echocardiograms, persistent Eustachian valves rarely result in clinical symptoms in newborns and infants without other significant structural heart disease. Previous reports have suggested that such patients require surgical treatment. The goal of this study is to delineate better the clinical course in infants presenting with symptoms that are a result of persistent Eustachian valves. A review of all patients with symptoms attributed to persistent Eustachian valves over a 10-year period at Riley Hospital for Children showed 3 of 4 patients improved, with eventual resolution of symptoms, with conservative management alone. This suggests that, contrary to previous reports, clinical observation may be warranted in many of these infants.

Cor Triatriatum↗